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ISSN 1392-3064

LIETUVOS NEUROLOGØ ASOCIACIJA LIETUVOS VAIKØ NEUROLOGØ ASOCIACIJA LIETUVOS NEUROCHIRURGØ DRAUGIJA

N e u r o l o g i j o s

9TH INTERNATIONAL CONFERENCE OF BALTIC CHILD NEUROLOGY ASSOCIATION (BCNA) JUNE 20­23, 2007 VILNIUS, LITHUANIA FINAL PROGRAM AND ABSTRACT BOOK 9-OJI TARPTAUTINË BALTIJOS ÐALIØ VAIKØ NEUROLOGØ ASOCIACIJOS KONFERENCIJA 2007 M. BIRÞELIO 20­23 D., VILNIUS, LIETUVA PROGRAMA IR TEZËS

2007, T. 11, Priedas Nr. 1

VILNIUS ,,ROTAS" 2007

NEUROLOGIJOS SEMINARAI SEMINARS IN NEUROLOGY

Leidþiamas nuo 1997 m. Steigëjas: VU MF Neurologijos klinika VYRIAUSIASIS REDAKTORIUS · EDITOR-IN-CHIEF Valmantas BUDRYS (Vilniaus universitetas) ATSAKINGOJI REDAKTORË · EXECUTIVE EDITOR Daiva RASTENYTË (Kauno medicinos universitetas) REDAKTORIØ KOLEGIJA · EDITORIAL BOARD Ilona BIÈKUVIENË (Vilniaus universitetas) Algirdas DEMBINSKAS (Vilniaus universitetas) Milda ENDZINIENË (Kauno medicinos universitetas) Egidijus JARÞEMSKAS (Vilniaus universitetas) Dalius JATUÞIS (Vilniaus universitetas) Alvydas JUOCEVIÈIUS (Vilniaus universitetas) Romas A. GVAZDAITIS (Kauno medicinos universitetas) Gintaras KAUBRYS (Vilniaus universitetas) Michel R. MAGISTRIS (Geneva, Switzerland) Diana OBELIENIENË (Kauno medicinos universitetas) Regina PARNARAUSKIENË (Vilniaus universitetas) Valius PAUZA (Kauno medicinos universitetas) Audrius V. PLIOPLYS (Chichago, IL, USA) Arûnas ÐÈIUPOKAS (Kauno medicinos universitetas) Juozas ÐIDIÐKIS (Kauno medicinos universitetas) Arimantas TAMAÐAUSKAS (Kauno medicinos universitetas) Nerija VAIÈIENË (Kauno medicinos universitetas) Reinhard VOLKMANN (Göteborg, Sweden) Walter-Uwe WEITBRECHT (Gummersbach, Germany)

Redaktoriø kolegijos adresas: Vilniaus universiteto ligoninës Santariðkiø klinikos Santariðkiø g. 2, LT-08661 Vilnius Tel./faks. (8 5) 236 52 20 El. p.: [email protected] http://www.neuro.lt

Editorial address: Vilnius University Santariðkiø Klinikos Hospital Santariðkiø 2, LT-08661 Vilnius, Lithuania Tel./fax (370 5) 236 52 20 E-mail: [email protected] http://www.neuro.lt

Leidinyje pateikiama informacija skirta tik specialistams medikams. Redakcijos nuomonë nebûtinai sutampa su straipsniø autoriø nuomone. Redakcija uþ reklamos turiná ir kalbà neatsako. Visi þurnale minimi vaistai turi bûti vartojami, atsiþvelgiant á naujausià vaistø vartojimo informacijà. ,,Neurologijos seminarø" leidybinës teisës yra leidëjo nuosavybë, saugoma autorinës teisës. Visos ar bet kurios þurnale spausdinamos medþiagos dalies dauginimui ir platinimui bet kokia forma ir priemonëmis ne asmeniniams tikslams bûtinas raðtiðkas leidëjo sutikimas. ,,Neurologijos seminarai" átraukti á Index Copernicus duomenø bazæ, kurioje referuojamø leidiniø moksliniai straipsniai pripaþástami tinkamais vertinant mokslo darbuotojø, kitø tyrëjø ir dëstytojø kvalifikacijà (Lietuvos mokslo tarybos 2006 m. balandþio 24 d. nutarimas Nr. VI-30) Kalbos redaktorë J. Niaurienë Maketavo V. Viluèio ámonë 2007 06 07. 6 spaudos lankai SL 021. Uþs. 208 Spausdino spaustuvë ,,Rotas", Pylimo g. 42, LT-01136 Vilnius © Neurologijos seminarai, 2007

TABLE OF CONTENTS

Patronage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4 Welcome of the Organizing Committee . . . . . . . . . . . . . . 5 Conference Organization . . . . . . . . . . . . . . . . . . . . . 7 Sponsors and Exhibitors . . . . . . . . . . . . . . . . . . . . . . 8 About the Conference . . . . . . . . . . . . . . . . . . . . . . . 9 Social Program . . . . . . . . . . . . . . . . . . . . . . . . . . 11 General Information. . . . . . . . . . . . . . . . . . . . . . . . 12 Scientific Program June 21 . . . . . . . . . . . . . . . . . . . . 13 Scientific Program June 22 . . . . . . . . . . . . . . . . . . . . 14 Scientific Program June 23 . . . . . . . . . . . . . . . . . . . . 16 Abstract Book . . . . . . . . . . . . . . . . . . . . . . . . . . . 17 Oral Presentations. . . . . . . . . . . . . . . . . . . . . . 18 Poster Presentations . . . . . . . . . . . . . . . . . . . . 30 Index of Authors . . . . . . . . . . . . . . . . . . . . . . . . . 41 List of Participants . . . . . . . . . . . . . . . . . . . . . . . . 45

Mrs. Alma Adamkienë, First Lady of the Republic of Lithuania is the patroness of the 9th International Conference of Baltic Child Neurology Association.

DEAR FRIENDS AND COLLEAGUES,

It is a great pleasure to welcome you at the 9th International Conference of Baltic Child Neurology Association (BCNA) in Vilnius on June 20-23, 2007. BCNA Conferences are held every other year and have turned out to be not just regional but also international forums for professionals interested in the improvement and development of care for children with neurological diseases. We are happy to declare the program of the 9th BCNA Conference which includes not only the usual "hot" topics of pediatric neurology like epilepsy or developmental disorders, but much attention is given to neurogenetic and neurometabolic diseases, also to the relatively new concept in our region ­ Orphan diseases and Orphan drugs. Interdisciplinary aspects of pediatric neurology will also be covered, including neuropsychiatry, or questions related both to pediatric neurology and neurosurgery like tumors, trauma, and hydrocephalus. A special lecture for young researches on preparation of publications is most valuable. Poster sessions will provide the opportunity of lively discussions with experts and followed by the nomination of the best poster. The geography of our delegates has expanded to 18 countries; the number of registered participants has exceeded 200. We are proud to have so many outstanding speakers with updated lectures and are happy to welcome all our colleagues and friends who are going to share their personal professional experience, both in formal and informal ways. Vilnius, the historical capital of Lithuania dating back to the 14th century, has the most beautiful and the largest Old Town, awarded with the status of World Cultural Heritage by UNESCO, with Vilnius University being the oldest one in Eastern Europe. It is rapidly expanding as a modern European capital, so please use the chance of experiencing the harmony of the old and the new Vilnius. For all of us this will provide beautiful atmosphere and the mood for work and friendship. Enjoy your stay in Lithuania Sveiki atvykæ! Welcome! With warmest regards,

Nerija Vaièienë Conference President

Milda Endzinienë Conference Vice-President

A high fat, low carbohydrate, nutritionally complete, powdered formula for the dietary management of drug resistant epilepsy in children over the age of one year. Formulated to induce and maintain ketosis. It is based on the classical 4:1 ratio (4g fat: 1g protein and carbohydrate). Convenient and easy to prepare as it is nutritionally complete - just measure and shake. Administer as a tube or sip feed either as a sole source of nutrition or meal replacement in a choice of flavoured and unflavoured 300g cans.

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SHS International office in Baltic countries: UAB Nutricia Baltics, Zirmunu str. 68, 124-Vilnius, Lithuania. Phone: (+370 5) 23 00 377 Fax: (+370 5) 23 00 404

CONFERENCE ORGANIZATION

Main organizers:

Lithuanian Child Neurology Association

Ministry of Health of the Republic of Lithuania Conference President Professor Nerija Vaièienë Ex-president and vice-president of Lithuanian Child Neurology Society President of Lithuanian Chapter of ILAE Department of Neurology Kaunas University of Medicine Eiveniu str. 2, LT-50009 Kaunas, Lithuania Tel. +370 37 326213 Fax. +370 37 326922 E-mail: [email protected] Conference Vice-President Professor Milda Endzinienë President of Lithuanian Child Neurology Society Ex-president and vice-president of Lithuanian Chapter of ILAE Department of Neurology Kaunas University of Medicine Eiveniu str. 2, LT-50009 Kaunas, Lithuania Tel. +370 37 326811 Fax. +370 37 326 852 E-mail: [email protected] Scientific Committee Nerija Vaièienë (Lithuania) Milda Endzinienë (Lithuania) Egils Vitols (Latvia) Tiina Talvik (Estonia) Local Organizing Committee Jûratë Grigonienë Rûta Praninskienë Tatjana Volkova Rûta Samaitienë Edita Kanytë Daiva Stankevièiûtë Conference Secretariat

Conbaltas UAB Baltic Conference Organizers ­ PCO Kareiviu str. 6, LT-09117 Vilnius, Lithuania Tel. +370 5 2120003 Fax +370 5 2120013 E-mail: [email protected] Website: www.bcna2007.com S7

ACKNOWLEDGEMENT

Organizing Committee of the 9th International Conference of Baltic Child Neurology Association is thankful for contribution of:

Gold Sponsor

Sponsors and Exhibitors

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

ABOUT THE CONFERENCE

Conference Dates June 20-23, 2007 Conference Venue The Conference will take place in Reval Hotel Lietuva. It is located on the beautiful bank of the river Neris in the center of Vilnius. Reval Hotel Lietuva Konstitucijos Ave. 20 Vilnius LT-09308, Lithuania Official Language English. No simultaneous translation will be provided. On-Site Registration Fees On site, in Euro Participant Resident and Student Accompanying Persons Gala Dinner One Day Participant Fee One Day Resident Fee Welcome Reception* 200 70 40 60 70 25 30 Certificate of Attendance Certificates of Attendance will be distributed on June 23. Abstract Book All the abstracts that have been accepted by the Scientific Committee are published in the scientific journal "Neurologijos Seminarai". Companies and delegates may copy materials for their personal use, but further copying for sale or for any other commercial purpose is prohibited without prior written permission by the Editor. Speakers' Room The speakers will be able to check their presentations in advance in the Speakers' Room which will be located in Epsilon hall. It will be open on: Thursday Friday Saturday June 21, 08:00 ­ 19:00 June 22, 08:30 ­ 18:00 June 23, 08:30 ­ 12:00 S9 Payment Payments on site should be made either in cash (Euro or Litas) or by credit card. Only registered and paid delegates can attend the Conference. Registration and Hospitality Desks The Registration and Hospitality Desks for the Conference will be located in the lobby of the Conference Center of Reval Hotel Lietuva. They will be open on: Wednesday Thursday Friday Saturday June 20, 15:00 ­ 18:30 June 21, 08:00 ­ 19:00 June 22, 08:30 ­ 18:00 June 23, 08:30 ­ 13:00

Conference Identification Badges The Conference Identification Badge will be included in the Conference material provided while registering for the Conference. There will be no admittance to the scientific sessions without the Conference Badge. Tickets to the social events will be collected on entry. The Conference Identification Badges will be also most helpful in contacts with other participants. Cost of replacing a lost or mislaid badge: 10 Euro.

* ­ charge is applied only for one day delegates. Registration Fee includes: · Attendance to all scientific sessions and the exhibition · Scientific documentation and a Conference bag · Welcome Reception · Coffee and lunches during the Conference · Certificate of attendance Accompanying Person's Registration Fee includes: · Welcome Reception · Vilnius City Tour One Day Registration Fee includes: · Attendance to that day scientific sessions · Scientific documentation and Conference bag · Coffee breaks and lunch during the Conference · Certificate of attendance

9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

The Speakers' Room is equipped with computers. Please note that the presentations should be downloaded in the Speakers' Room. All the speakers are kindly requested to provide the electronic versions of their presentations one day prior to the session (especially important for video presentations). Exhibition A commercial and technical exhibition will form an integrated part of the Conference. It will take place in the lobby of the Conference Center of Reval Hotel Lietuva. Opening Hours: Thursday Friday Saturday

The posters should be brought in person and put up on Wednesday June 20, 15:00 ­ 18:30 The posters should be taken off Saturday June 23, 13:00 ­ 14:00 Posters that will not be taken off by the specified deadline will be destroyed. During the Poster Exhibition on 22 of June the best poster will be selected and awarded! Internet Corner The Internet Corner will be located in the lobby of the Conference Center of Reval Hotel Lietuva. It is free of charge and it will be open during all Conference hours. Coffee Breaks Coffee, tea and other refreshments will be served in the exhibition area as well as in the lobby of the Conference Center of Reval Hotel Lietuva. Lunches Lunches will be served in restaurant "River Side" at the Reval Hotel Lietuva.

June 21, 08:00 ­ 19:00 June 22, 08:30 ­ 18:00 June 23, 08:30 ­ 12:00

Poster Exhibition The Poster Exhibition will take place in the lobby of the Conference Center of Reval Hotel Lietuva. The Poster Exhibition will open on: Thursday June 21, 08:00 ­ 19:00 Friday June 22, 08:30 ­ 18:00 Saturday June 23, 08:30 ­ 12:00

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

SOCIAL PROGRAM

THE WELCOME RECEPTION June 20, Wednesday. 19:00 Free for participants and accompanying persons* A Welcome Reception followed by tender music will take place in the Reval Hotel Lietuva, Beta Hall ­ Conference venue, (Konstitucijos Ave 20), situated on the right bank of the river Neris, in the center of Vilnius. Luscious food and refreshing drinks will set everyone for a pleasant evening. This is perfect time to meet old colleagues and make new contacts. Dress code ­ smart casual. *Entrance fee for One Day Participants is 30 Euro. GALA DINNER AT BELMONTAS ENTERTAINMENT CENTER June 22, Friday. 20:00 Entrance fee is 60 Euro A Gala Dinner will take place in the remarkable Belmontas Entertainment Center, at the restaurant ­ mill of the Carl de Vimo, located in the picturesque surroundings of Vilnius. Romantic pedestrians path will give you pleasure for a walk in the fresh air by the river under the starlight sky. Musical orchestra "Mezzo" will play unforgettable Frank Sinatra and Elvis Presley time music together with outstanding Lithuanian soprano Auðra Liutkutë. Comfortable busses will pick you up and bring to the restaurant. Delicious food and tender music will treat everyone for the beautiful moments. Dress code ­ smart casual. VILNIUS CITY TOUR June 22 Friday. 13:00 Free for accompanying persons Vilnius City Tour continues for 2.5 hours (on foot) and takes you through the most interesting places in Vilnius Old Town. Old Town is the largest in Eastern Europe. Vilnius has always attracted visitors and fascinated everyone with its architectural diversity. The ruins of castles, an old network of narrow streets, church spires, bell towers, red tile roofs, residential cellars... Excursion starts at 13:00 at the Cathedral Square. The tour is included in the registration fee of accompanying persons (please pick up your tickets at the Hospitality Desk); for others price per person is 15 Euro.

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

GENERAL INFORMATION

About Lithuania Lithuania is the largest and southernmost of the Baltic States. Lithuania is bordered by Latvia to the north, Belarus to the east and south and Poland and Russian Federation to the south and southwest. Forest spread through almost 30 percent of the country, and rivers, streams and more than 3000 lakes shape the land. Five national and 30 regional parks preserve this paradise for boating, fishing, hunting, bird watching or horseback riding. The largest river, Nemunas, flows into the Curonian Lagoon separated from the Baltic Sea by the Curonian Spit. This 98 km long bank of sand, dunes and pine trees stretches from Kalingrad in the south to the seaport of Klaipeda in the north. The Curonian Spit recognized by UNESCO as a part of the world heritage, is one of the most unspoiled natural spots in the whole Baltic Region. It is also known as the amber coast, for the precious amber that was washed onto this shore for many centuries. About Vilnius The capital, Vilnius, is one of the most beautiful towns in Eastern Europe, situated where the Neris and Vilnele rivers meet and surrounded by

picturesque wooded hills. Founded in 1323, its unique character and architecture reflects its history at crossroads of the Roman, Byzantine and Eurasian worlds. Vilnius' many churches and towers, fortifications, secluded medieval courtyards and narrow streets are a mix of Gothic, Renaissance, and Baroque and Classical styles. Vilnius, like all Lithuanian cities, has a wide selection of restaurants. Lithuanian beer is widely considered to be some of the best in the world. Drink it with tasty local breads and cheeses or national dishes like "zeppelins", balloon-shaped potato cakes filled with meat, or potato sausages and pancakes. In 2009 Lithuania will celebrate a full millennium since the first mentioning of its name in historical sources. Climate Lithuania has a central European climate. The end of June generally is warm. Take a sweater for a cool evening and an umbrella for a shower. Time Eastern Europe Summer time 3 hours ahead of Greenwich Mean Time. Bus Services Vilnius has an effective and cheap network of busses and minibuses. Prices for buses and minibuses are up to 1 Euro. Taxi Numerous taxis are operating in Vilnius. Taxis hired in street can be expensive and not always of the best quality. It is recommended to call Martono Taxi 1422 or +37052400004. The price for a taxi from the airport to the center is around 15 Euros.

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

PROGRAM

20 June, Wednesday

15:00 ­ 18:30 Registration 19:00 Welcome Reception

21 June, Thursday

8:30 ­ 9:20 9:20 ­ 11:00 Opening of the Conference Morning session I: PERINATAL NEUROLOGY AND DISABILITY Chairing: Tiina Talvik (Estonia), Eugen Boltshauser (Switzerland) Brigitte Vollmer (Sweden) "Follow up of preterm children: relationship between brain structure and clinical outcome" 9:50 ­ 10:20 Michel Magistris (Switzerland) "Obstetric brachial plexus palsy: old problem, new understanding" 10:20 ­ 10:45 Anita Vetra (Latvia) "Spasticity in children" 10:45 ­ 11:00 Laima Mikulënaitë, Rûta Samaitienë, Liuda Istigeèeva (Lithuania) "Improvement of functional abilities in children with cerebral palsy treated with botulinum toxin" 11:00 ­ 11:30 Coffee break 11:30 ­ 13:00 Morning session II: BETWEEN NEUROLOGY AND NEUROSURGERY Chairing: Egils Vitols (Latvia), Vytautas Ragaiðis (Lithuania) 11:30 ­ 12:00 Liana Beni-Adani (Israel) "The occurrence of obstructive versus absorptive hydrocephalus in newborns and infants: relevance to treatment choices" 12:00 ­ 12:15 Dovilë Grinkevièiutë, Vytautas Ragaiðis (Lithuania) "Intracranial pressure-targeted treatment of severe pediatric head injury: Lithuanian experience" 12:15 ­ 12:30 Inga Talvik, Mairi Mannamaa, Tiina Talvik (Estonia) "Inflicted traumatic brain injury or shaken baby syndrome in Estonia: incidence and long-term outcome" 12:30 ­ 12:45 Rosita Kiudelienë, Giedrë Rutkauskienë, Algimantas Matukevièius, Donata Jurgaitytë, Lingvita Gumbelevièienë, Nerija Vaièienë (Lithuania) "Pediatric brain tumors in Lithuania: epidemiological overview" 12:45 ­ 13:00 Algimantas Matukevièius (Lithuania) "Brainstem gliomas in children" 9:20 ­ 9:50

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

13:00 ­ 14:00 Lunch 14:00 ­ 16:00 Afternoon session I: EPILEPSY I Chairing: Rochelle Caplan (USA), Ulrich Stephani (Germany) 14:00 ­ 14:30 Olivier Dulac (France) "Metabolic diseases with epilepsy" 14:30 ­ 15:00 Marina Nikanorova (Denmark) "Epilepsies of childhood: diagnostic challenges" 15:00 ­ 15:30 Walter Van Emde Boas (The Netherlands) "Frontal lobe epilepsies in childhood: semiology and clinical spectrum" 15:30 ­ 15:45 Nebojsa Jovic (Serbia) "Malformations of cortical development: is prognosis of epilepsy predictable?" 15:45 ­ 16:00 Jurgis Strautmanis (Latvia) "Should we treat epilepsy?" 16:00 ­ 16:30 Coffee break 16:30 ­ 18:30 Afternoon session II: NEUROPSYCHIATRY AND COGNITIVE DISORDERS Chairing: Lene Sahlholdt (Denmark), John Stephenson (Scotland) 16:30 ­ 17:00 Sigita Plioplys (USA) "Current views on pediatric psychogenic non-epileptic seizures" 17:00 ­ 17:30 Rochelle Caplan (USA) "Diagnosis and feedback in pediatric non-epileptic seizures: do's and don'ts" 17:30 ­ 17:50 Aurelija Juèaitë (Sweden) "Attention deficit hyperactivity disorder: alterations of dopaminergic system" 17:50 ­ 18:10 Darius Leskauskas (Lithuania) "Prevalence and current treatment of attention deficit ­ hyperactivity disorder in Lithuania primary school pupils" 18:10 ­ 18:30 Anneli Kolk (Estonia) "Cognitive dysfunction of children with symptomatic and newly diagnosed epilepsy"

22 June, Friday

8:30 ­ 10:30 Morning session I: EPILEPSY II Chairing: Nerija Vaièienë (Lithuania), Walter van Emde Boas (The Netherlands) Perrine Plouin (France) "Seizure semiology and syndromic approach in children under 3 years" 9:00 ­ 9:30 Athanasios Covanis (Greece) "Panayiotopoulos syndrome vs Gastaut-type occipital epilepsy" 9:30 ­ 10:00 Rochelle Caplan (USA) "Psychiatric and cognitive comorbidities in pediatric epilepsy: cause or effect?" 10:00 ­ 10:30 Sigita Plioplys (USA) "Pharmacological treatment of attention deficit hyperactivity disorder in children with epilepsy" S14 8:30 ­ 9:00

9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

10:30 ­ 11:00 Coffee break 11:00 ­ 13:00 Morning session II: GENETIC AND DEGENERATIVE DISORDERS Chairing: Birutë Skerlienë (Lithuania), Leo Spaapen (The Netherlands) Ulrich Stephani, Hiltrud Muhle, Sarah von Spiczak (Germany) "Current achievements in the genetics of the epilepsies" 11:30 ­ 12:00 Eugen Boltshauser (Switzerland) "Cerebellar atrophies" 12:00 ­ 12:30 Lina Basel - Vanagaitë (Israel) "Genetics of mental retardation: present and future" 12:30 ­ 13:00 Thomas Sejersen (Sweden) "Future treatments of neuromuscular disorders" 13:00 ­ 14:00 Lunch 14:00 ­ 15:30 Afternoon session I: RARE DISEASES AND ORPHAN DRUGS Chairing: Milda Endzinienë (Lithuania), Thomas Sejersen (Sweden) 14:00 ­ 14:25 Leo Spaapen (The Netherlands) "Inherited metabolic causes of neurological diseases in neonates and infants" 14:25 ­ 14:50 Arrigo Schieppatti (Italy) "Orphan drugs and rare diseases: problems and opportunities" 14:50 ­ 15:15 Paola Baiardi, Manuel de La Paz, Lars Savendahl, Domenica Teruscio, Renza Barbon Galluppi, Enrico Morten, Donatas Stakisaitis, Adriana Ceci (Italy) "Accessing information on orphan drugs: the EuOrphan Service" 15:15 ­ 15:30 Indrë Ðpokienë (Lithuania) "Orphan patients and orphan medicines: situation in Lithuania" 15:30 ­ 16:00 Coffee break 16:00 ­ 16:45 SPECIAL TOPIC! Roger A. Brumback (USA) "Publishing in biomedical journals: hints for success from a journal Editor" 16:45 ­ 17:30 POSTER SESSION Moderators: Marina Nikanorova (Denmark), Michel R. Magistris (Switzerland), Athanasios Covanis (Greece) 20:00 Gala Dinner 11.00 ­ 11:30

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

23 June, Saturday

9:00 ­ 10:30 Morning session I: EPILEPSY AND DISABILITY Chairing: Sigita Plioplys (USA), Nebojsa Jovic (Serbia) Jurgita Grikinienë (Lithuania) "Epilepsy, magnesium and gender differences" 9:15 ­ 9:30 Ljerka Cvitanovic Sojat, Masa Malenica, Romana Gjergja, Zlatko Sabol, Tina Sojat (Croatia) "Febrile seizures and epilepsy: a retrospective study" 9:30 ­ 9:45 Ulvi Vaher, Aita Napa, Tiina Talvik (Estonia) "Seizures and value of EEG investigations in asphyxiated term newborn infants" 9:45 ­ 10:00 Jûratë Grigonienë, Dovilë Raèkauskaitë, Milda Endzinienë, Nerija Vaièienë (Lithuania) "Neurodevelopmental outcome of neonatal seizures" 10:00 ­ 10:15 Svajûnë Gradeckienë, Danguolë Ostrauskienë, Lina Norkienë, Asta Kutraitë, Milda Endzinienë, Nerija Vaièienë (Lithuania) "Treatment of spasticity with botulinum toxin A in cerebral palsy: three years experience at Kaunas University Hospital" 10:15 ­ 10:30 Ugur Cavlak, Naile Guney (Turkey) "Physical characteristics of unaffected limbs of cerebral palsied children" 10:30 ­ 11:00 Coffee break 11:00 ­ 12:30 Morning session II: MISCELLANEOUS Chairing: Guntis Rozentals (Latvia), Aurelija Juèaitë (Sweden) 11:00 ­ 11:15 Birutë Skerlienë, Jûra Tulevièienë, Marija Jakutoviè, Donatas Petroðka (Lithuania) "Muscular presentation of inherited disorders of mitochondrial metabolism in children" 11:15 ­ 11:30 Nerija Vaièienë (Lithuania) "Juvenile onset Huntington disease" 11:30 ­ 11:45 Milda Endziniene (Lithuania) "A case of severe Pelizaeus-Merzbacher disease caused by the triplication of PLP1 gene" 11:45 ­ 12:00 Rûta Praninskienë, Aurelija Juèaitë, Irena Dumalakienë, Mykolas Mauricas (Lithuania) "Melatonin secretion in children: developmental and neurological disorders" 12:00 ­ 12:15 Rûta Samaitienë, Laima Mikulënaitë, Liuda Istigeèeva (Lithuania) "Sleep disorders in children with developmental disabilities" 12:15 ­ 12:30 Karlis Kupcs, Helmuts Kidikas, Igors Aksiks, Svetlana Rudnicka, Aleksejs Vinogradovs, Janis Savlovskis (Latvia) "Endovascular treatment of intracranial aneurysms in children ­ first experience" 12:30 Closing of the Conference 9:00 ­ 9:15

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ABSTRACT BOOK

9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

ORAL PRESENTATIONS 21 June, Thursday

FOLLOW-UP OF PRETERM CHILDREN: RELATIONSHIPS BETWEEN BRAIN STRUCTURE AND CLINICAL OUTCOME

Brigitte Vollmer

Karolinska Institute, Stockhol, Sweden

Infants born prematurely are at high risk of long term neurological and neurodevelopmental impairments. About 10­15% of those born at < 32 weeks of gestation will have major impairments such as cerebral palsy, severe visual impairment, global cognitive delay and/or epilepsy. Brain lesions in preterm children are typically located in the periventricular white matter. It has been shown that there is a relationship between location and extent of these lesions as seen on conventional structural magnetic resonance imaging (MRI) and outcome as far as the major impairments are concerned. Up to 30% of those preterms who survive without major impairments will have minor neurological signs, specific cognitive deficits, attentional and behavioural difficulties and these minor impairments have been shown to have significant implications for e.g. educational progress of children born prematurely. It has been suggested that these deficits are partly caused by subtle structural brain abnormalities that may be too subtle to be identified by purely visual analysis of MR images. Indeed results from recent studies that have used sophisticated MRI acquisition and analysis techniques indicate that subtle structural brain abnormalities are associated with some of the minor difficulties seen in children born preterm. In this presentation I will discuss both the relationships between major impairments and brain abnormalities and associations between minor impairments and subtle structural brain abnormalities.

Electrophysiology has demonstrated the major role in sequel of OPP of faulty reinnervation causing simple misdirection (axon that reinnervates wrong muscle) and complex misdirection (axon sprouts that invades 2 or several motor or sensory nerves). Understanding of these phenomena is mandatory to explain the particular condition of a patient and to envisage the appropriate treatment. Surgery started at the beginning of the 19th century and continued until 1930. It was stopped due to poor results and replaced by conservative measures which are similarly inefficient. Attempts using microsurgical techniques are being undertaken but efficient management remains a challenging question. Selection of patients most likely to benefit from microsurgical intervention may derive from scrupulous electrophysiological and non-invasive functional tests.

SPASTICITY IN CHILDREN

Anita Vetra

National Rehabilitation Center, Jurmala, Latvia

OBSTETRICAL BRACHIAL PLEXUS PALSY: OLD PROBLEM, NEW UNDERSTANDING

Michel R. Magistris

Geneva University Hospital, Geneva, Switzerland

Description of brachial palsy of infants dates back to Hippocrates time. Relation to birth and obstetrics is first considered in the mid-1700. The term Obstetrical Brachial Plexus Palsy (OPP) is proposed in 1872. OPP relates to traction on the brachial plexus during delivery causing brachial palsy. Extension of the palsy may concern roots C5-C6, C5-C7, C8, Th1. Recovery depends on the 3 possible types of nerve injury. Extremes are represented by neurapraxia (conduction block caused by compression) that recovers completely in less than 3 months (most frequent situation), and by neurotmesis or root avulsion which have no spontaneous potential to recover. Recovery from axonotmesis depends on the proportion of dis rupted ax ons. If reinnervation by col lat eral i n t r a - m u s c u l a r s p r o u t i n g i s i n s u f fi c i e n t , t e r m i n a l reinnervation occurs that may lead to incomplete and faulty reinnervation. This ex plains (i) per sis tent weak ness, (ii) co-contractions (abnormal syncinesis) restricting intended movements, and (iii) therapeutic limitations. Evaluation of extension, type of nerve fiber damage and subsequent re gen er a tion re quires care ful clin i cal, ra dio log i cal and electrodiagnostic investigations. The latter are difficult to apply to in fants but should be con sid ered at later age.

Clinical expierence with botulinum toxin A (BtA) in the treatment of spasticity in children started in early 1990s with the first trials reported by Koman and Graham and coworkers. Several studies have documented efficacy, safety and sideeffects. BtA injections result in focal, temporary chemodenervation of muscles, which can lead to functional gains. The expression `spastic motor disorder' is used as an umbrella term. It refers to complex disorders involving cortical, spinal and musculosceletal systems. The most common clinical syndrome is cerebral palsy (CP). Spasticity is only one component of the upper motorneurone syndrome. Whatever the cause, local administration of BtA may temporarily reduce muscular hyperactivity. There are several important questions when consider treatment with BtA: suitability of local therapy, indications for treatment, patient selection, timing of treatment, interdisciplinary treatment- combination therapy. There are many published studies reported evidence of benefits from using BtA in pediatric spasticity. BtA improve posture, spasms, function and ease of care, and may reduce the need for other tretment. In the last few years several randomised control trials of BtA for spastic equinus have been completed. Many experts believe that BtA is helpful for arm spasticity, but controlled data come only from few small studies and more trials are needed. The most common deformity in CP is spastic equinus and the most frequent indication for the use of BtA is dynamic equinus, but management is complicated by the fact that not every child has a true equinus gate. The goals of managing spastic equinus vary from facilitating independent walking to better tolerance of orthosis. Evaluation of the outcome is essential, because treatment with BtA is a relatively new. BtA treatment should be given as a part of spasticity service and should not be used in isolation.

IMPROVEMENT OF FUNCTIONAL ABILITIES OF CHILDREN WITH CEREBRAL PALSY TREATED WITH BOTULINUM TOXIN

Laima Mikulenaite, Ruta Samaitiene, Liuda Istigeceva

Vilnius University Children Hospital Child Development Center, Vilnius, Lithuania

Introduction: Botulinum toxin A (Dysport) is being used for treatment of children with spasticity to reduce impairment and functional limitations. Previously for many years treatment of cerebral palsy has centered on the use of physiotherapy and orthotics and some medications to overcome the problems of leg spasticity.

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

Purpose: To evaluate the effect of treatment of children with spastic cerebral palsy to functional limitations associate with disability in daily life. Patients, methods: 54 patients (age range 2­9 years), treated in Child Development Center, Vilnius, Lithuania with spastic cerebral palsy were enrolled. The outcome measure was observational gait analysis, the modified Ashworth Scale, range of motions, Selective Motor Control Scale, Gross Motor Function measure (GMFM-66), sitting scale and functional abilities scale. The indication for injection was the presence of a dynamic contracture of lower-limb muscles interfering with positioning or walking. Spastic target muscles were identified by clinical examination. The effects of injection were monitored by repeated clinical examination. Results: Improvements have been documented in tone reduction, range of motion, gait pattern, sitting and functional abilities interfering with daily life of patient.

INTRACRANIAL PRESSURE TARGETED TREATMENT OF SEVERE PEDIATRIC HEAD INJURY: LITHUANIAN EXPERIENCE

Dovile Grinkeviciute, Vytautas Ragaisis

Kaunas University of Medicine, Kaunas, Lithuania

Background. Despite preventive measures traumatic brain injury remains the major injury-related mortality and morbidity factor among children. Outcome is a consequence of the initial impact and secondary injury mecanisms. Intracranial pressure targeted therapy is based on physiological principles for volume regulation of the intracranial compartment. The essential goal of this therapy is to maintain cerebral perfusion pressure (CPP) above certain level. In Pediatric Intensive Care Unit (PICU) of Kaunas Medical University Clinic (KMUC) we started applying the protocol of severe head injury treatment in 2003. Method. This was a retrospective study of all patients after severe head injury treated over a period of 1999­2006. We compared the outcomes after severe head injury before and after 2003 (1-st and 2-nd groups) on discharge and after 6 months using Glasgow Outcome Scale (GOS). Head injury was defined as severe if postresuscitation Glasgow Coma Scale (GCS) £ 8. Poor outcome referred to GOS £ 3, good outcome referred to GOS > 3. Results. 137 children after severe head injury were involved in the study. Intracranial pressure (ICP) was measured in 4 (6,5%) cases in first group and in 36 (48%) in second group. Decompressive craniectomy was performed in 23 (37,1%) and 32 (42,1%) cases. Mortality rate was 33,9% in the first and 29,9% in the second group. Totally 95 (69,3%) patients survived, and outcomes were evaluated only for survivals. On discharge poor outcome was applied to13 (31.7%) and good outcome to 28 (68.3%) patients in the first group and subsequently 26 (48.1%) and 28 (51%) patients in the second group. The difference between the groups was not statistically significant (p>0.05). But after 6 months the difference was statistically significant: 8 (19.5%) and 33 (80.8%) for the first group and 3 (5.6%) and 51 (94.4%) for the second group (p<0.05) Conclusions.Treating pediatric patients according intracranial pressure targeted therapy results in a favourable long-term outcome.

THE OCCURRENCE OF OBSTRUCTIVE VERSUS ABSORPTIVE HYDROCEPHALUS IN NEWBORNS AND INFANTS: RELEVANCE TO TREATMENT CHOICES

Liana Beni-Adani, Naresh Biani, Liat Ben-Sirah, Shlomi Constantini

Dana Children's Hospital, Tel-Aviv Sourasky Medical Center, Israel

Objective: The classification of hydrocephalus in newborns and in infants is different from the classification in adulthood. The objective of this paper is to present the spectrum of obstructive-communicating Hydrocephalus, which is more complex in the pediatric group, and to propose the relevance of this particular classification to treatment options. Materials and Methods: The authors categorized infants with active hydrocephalus at time of presentation into the following 4 groups along the spectrum of communicating versus obstructive HCP Group 1: Patients with a purely ab. sorptive (communicating) HCP In these patients, 4-ventricu. lar dilatation is usually observed, with occasional extra-axial fluid accumulation. An extra-cranial CSF diversion (shunt) is the treatment of choice. Group 2: Patients with an obstructive component together with a persistent absorptive component. In these patients, a technically successful endoscopic procedure will not prevent progression of clinical symptoms of HCP An extra-cranial CSF diversion (shunt) . should be the treatment of choice, even though some of these patients are currently treated by endoscopy. Group 3: Patients with an obstructive component together with a temporary absorptive component. In these patients, a technically successful ETV should be followed by temporary CSF drainage. Group 4: Patients with a purely obstructive HCP In . these patients, an endoscopic procedure (ETV) is the treatment of choice. Results: The data suggests that obstructive hydrocephalus in the very young population may be rather a combination of obstructive and absorptive problem. The outcome of the patient depends mainly on the basic pathology causing the hydrocephalus but also on the treatment that is chosen and its complications. In transient absorptive hydrocephalus, temporary measures were effective in many cases leading to successful procedures of ETV and / or posterior-fossa decompression in selected cases. Conclusions: Understanding the basic pathology in a given child may open the window of opportunities for other than shunt surgery in many hydrocephalic children with major obstructive component.

INFLICTED TRAUMATIC BRAIN INJURY (ITBI) OR SHAKEN BABY SYNDROME (SBS) IN ESTONIA: INCIDENCE AND LONG-TERM OUTCOME

Inga Talvik, Mairi Mannamaa, Tiina Talvik

Children's Clinic of Tartu University Hospitals, Tartu, Estonia

Inflicted traumatic brain injury (ITBI) or shaken baby syndrome is recognized as a major cause of disability and death in the paediatric population. The aim of the study was to investigate the incidence rate of ITBI in Estonia and long-term outcome Patients and methods During the period from January 1st 1997 and December 31st 2003 a total of 26 children met the criteria of ITBI: 4 children died, 22 survived. All survivors were examined during the years 2005­2006 by the team of: neurologist, psychologist and an ophthalmologist. Results: The study group in incidence study included 26 children, 20 (77%) were boys and six (23%) were girls, 4 (15,4%) of them died Median age at admission to hospital was 3.9 mo, and the boys were younger than the girls. The overall incidence of ITBI was 28.7 per 100,000 infants. In the prospective group the incidence was 40.5 per 100,000, and in retrospective group 13.5 per 100,000. The study group for follow up included all 22 children, 18 (82%) boys, 4 (18%) girls. The mean

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

age of chil dren at fol low-up was 5,2 years (range 2,3­9,0 years). The follow ­ up period was 4,67 years (range 2,3­8,6 years) Severe motor problems were in 5/22 children (22%), 13/22 had light to moderate neurological symptoms. One child was autistic. Epilepsy was diagnosed in 7/22 cases (32%). Only 2/22 (9,1%) children were without any developmental problems at the follow-up. 17/22 (77%) had delayed mental development. In conclusion. The incidence of ITBI in Estonia was 40.5 per 100,000. Only 2/22 children were without any problems in follow-up and 20/22 had problems with different severity. More educational programs for prevention of abusive behavioural of parents. ogy is complex or inappropriate. Some proportion of epileptic syndromes end up being classified in non-specific categories ­ "cryptogenic lo cal iza tion-re lated epilepsies" or "epilepsies without unequivocal generalized or focal features". It should be taken into account that many epileptic syndromes are common and well defined, others need further research and categorization. There are as well syndromes where genetics and pathophysiology have been dramatically clarified (BFNC), some new single-gene syndromes described in the recent years and new syndromes reported only by several investigators (migrating focal seizures of early infancy). Another pitfall of the current classification is terminology which not always is relevant. The dichotomic subdivision into "localization-related" versus "generalized" epilepsies might be difficult in some cases, as there are rather many conditions lying between these 2 categories (encephalopathy with CSWS). Term "focal" should be used in respect of seizures but not syndromes (all patients with localization-related epilepsies have focal seizures but not all have focus ­ BECTs). The other challenge is to determine to what extent we have to split epilepsies into different syndromes.

PEDIATRIC BRAIN TUMORS IN LITHUANIA: EPIDEMIOLOGICAL OVERVIEW

Rosita Kiudeliene, Giedre Rutkauskiene, Algimantas Matukevicius, Donata Jurgaityte, Lingvita Gumbeleviciene, Nerija Vaiciene

Kaunas University Hospital, Kaunas, Lithuania

Background. Brain tumors are the most common solid tumors that occur in children. Up to date there is no published epidemiological data on pediatric brain tumors in Lithuania. Aim of the study was to analyze incidence, morphological type, treatment modalities and mortality rate in children with brain tumors. Methods. We retrospectively analyzed medical records of 171 cases of primary brain tumors that within seven years period (2000­2006) were diagnosed and treated at Kaunas Medical University Hospital which serves as the only hospital dealing with pediatric neurooncology in Lithuania. Results: The incidence of brain tumors was 16­32 new cases per year. The age of patients ranged between 10 months­ 17 years (mean 8.68±4.97 years), 93 (54.4%) were boys, 78 (45.6%) were girls. Low grade gliomas constituted 25%, medulloblastomas/PNET ­ 23%, high grade gliomas ­ 20% of all brain tumors. Radical surgery was performed in 32.9%, partial in 34.7% of cases, biopsy only were taken in 15.3% of cases. Surgery was not performed in 17.1% of patients: 5.3% were given chemotherapy, 11% ­ radiotherapy only. 23.4% of children with brain tumors received complex management. 25.7% of patients died within 1­84 months (mean 38.2±25.8 months) period since the moment of diagnosis, 66.7% of patients survived, mainly with low grade gliomas, germ cell tumors and craniopharyngeomas, 25.7% of patients died: 76.9% with brainstem tumor, 60.9% with medulloblastoma/ PNET, 60% with high grade gliomas. Conclusions. Incidence of brain tumors in Lithuania is 3/100.000; male-female ratio is 1.2:1. Low grade gliomas are the most common malignancies causing low death rate. Complex management of brain tumors in Lithuania is still not sufficient due to recent introduction of chemotherapy.

FRONTAL LOBE EPILEPSIES IN CHILDHOOD: SEMEIOLOGY AND CLINICAL SPECTRUM

Walter van Emde Boas

Stichting Epilepsie Instellingen Nederland, Heemstede, The Netherlands

EPILEPSIES OF CHILDHOOD: DIAGNOSTIC CHALLENGES

Marina Nikanorova

Danish Epilepsy Center, Dianalund, Denmark

Besides the rather clear-cut guidelines of ILAE Classification of Epilepsies and Epileptic Syndromes, their diagnosis in childhood is often a challenge. It might be due to several reasons: some patients cannot be given a recognized syndrome diagnosis; syndromes overlap or evolve one from another; syndromes change as new information is obtained; terminol-

Frontal lobe epilepsies are defined as localisation related epilepsies or epilepsy syndromes characterised by simple or complex partial and/or secondary generalised seizures emanating from the frontal lobe. They constitute a heterogeneous group of seizure disorders characterised by a variety of seizure types, EEG patterns and aetiologies. Their exact prevalence is unknown but they may constitute up to 1/3 of all epilepsies in adults and probably as much or even more in children. Up to seven different types of frontal lobe epilepsy are recognised in the 1989 ILAE classification of epilepsies and epileptic syndromes, dependent on their (presumed) area of seizure onset within specific area's of the frontal lobes and on specific ictal signs and symptoms, known to relate to ictal activity in that specific area. Most are characterised by predominant motor signs, sometimes involving massive and at times bizarre hypermotor behaviour which may suggest nonepileptic or psychogenic behaviour, but behavioural arrest (mimicking absence seizures), autonomic signs and symptoms and emotional changes, notably fear, may be an expression of frontal lobe seizures. Due to the intricate neuronal networks connecting the frontal lobe to other brain areas and within the frontal lobe itself and due to the propensity of very fast propagation of ictal activity through these networks, the assessment of these epilepsies is not always easy, especially since the MRI in many of these patients is negative and the EEG often is relatively non-contributory, may be misleading or can actually suggest generalised epilepsy. In children the differential diagnosis is further hampered by the fact that `typical' frontal lobe or temporal lobe semeiology is often lacking during the first decade. Frontal lobe seizures can present with `typical' bilateral spike and wave patterns, at times making differential diagnosis with idiopathic generalised epilepsies extremely difficult. Moreover there is increasing evidence that at least some of the archetypal idiopathic generalised epilepsies, including childhood absence epilepsy, express themselves in seizures which involve relatively limited neuronal circuits, including the frontal cortex and some thalamic nuclei, rather than the hemispheric cortex as a whole. The clinical spectrum of frontal lobe epilepsy thus is evolving and may come to include epilepsy syndromes that, at this point, are considered to be generalised rather than localised.

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

MALFORMATIONS OF CORTICAL DEVELOPMENT: IS PROGNOSIS OF EPILEPSY PREDICTABLE?

Nebojsa Jovic

Clinic of Neurology and Psychiatry for Children and Youth, Belgrade, Serbia

Malformations of cortical development (MCD) are often recognized as underlying etiology for intractable seizures in children. Early prediction of clinical course and identification of pharmacoresistance are challenge in their management. Methods: A Group of 212 children and adolescents aged 0.5 to 17 years with MCD was prospectively studied (3­9.5 years) for clinical course, prognosis and seizure control. Various MCD were disclosed by MRI techniques and classified (Barkovich, Brain Dev 2002; 24: 2­12). Results: Seizures occurred in 167 (78.8%) MCD patients with mean seizure onset of 3.6 years. Mental retardation was found in 61.3% of children. Focal neurologial deficits were noted in 91 patients. Generalized epileptic syndromes were diagnosed in 64, partial epilepsies in 103 patients. Epileptic status occurred in 17.9% epileptics. Seizures refractory to AEDs occurred in 43.1% patients (Group A). Complete long-term seizure control was achieved in 18.6%, till >75% seizure reduction was noted in 38.3% (Group B). New AEDs were effective in 56 patients. MCD with multilobar involvement, hemimegalencephaly, lyssencephaly 1, subcortical heterotopias and perysylvian polymicrogyria were more often associated with intractable epilepsy in comparison with partial schizencephaly, focal CD, TSC and unilateral pahygyria. Seizure onset was significantly earlier in a Group A (mean 2.3 yrs) when compared with Group B (7.2 yrs). Infantile spasms occurred in 13.8% of children with later pharmacorestance vs. 4.8% of children from Group B. Favourable initial therapeutic response was significantly higher in a Group B (58/95) vs. Group A (16/72). Pharmacoresistant seizures were most often associated with mental retardation, sensory impairment and undeveloped speech before epilepsy and multifocal/bilateral EEG abnormalities when compared with well-controlled patients. Conclusions: Early seizures onset, occurrence of infantile spasms, poor response to initial treatment, multilobar/bilateral MCD and early development of severe clinical condition were identified as main predictors of seizure intractability.

zures as reflex reaction to hypoxia. G. Doman, R. Pelligra added to the theory novel data about relationship of neuronal activity and cerebral blood flow regulation, role of ATP dependent ion pumps in the ictogenesis and the fact that there is hyperperfusion, hypermetabolism during seizures and hypoperfusion, hypometabolism in interictal period in epileptogenic region. If we look at such approach critically we can find little data in the literature that seizures could be beneficial. On the contrary there are lot of reports that seizures can induce brain lesion, predicts higher trauma and mortality risk, is associated with social and psychiatric problems and this is true even in idiopathic epilepsy despite difficulties to separate effects of underlying diseases and seizures. Conclusions. I suppose presented theory of ictogenesis is interesting and demands further discussion in scientific literature. Unfortunately it is used in malicious manner in IAHP . There is little data that seizures could be beneficial but lot of data that seizures could be harmful.

CURRENT VIEWS ON PEDIATRIC PSYCHOGENIC NON-EPILEPTIC SEIZURES

Sigita Plioplys

Children's Memorial Hospital, Chicago, USA

Objective: To review current research data on epidemiological, clinical and developmental characteristics of pediatric psychogenic non-epileptic seizures (PNES). Background: Children with PNES are often undiagnosed and untreated, because of the difficulties in timely diagnosis and lack of standardized treatment guidelines. While searching for the "correct" diagnosis, these patients are frequently exposed to unnecessary medical procedures and treatments, which may cause iatrogenic side effects, significant economical burden to the families and high utilization of health care services. Methods: Data on prevalence, risk factors, clinical and developmental characteristics, comorbid psychopathology and long term outcome of pediatric PNES will be discussed. Psychodynamic, Behavioral, and Family Systems theories of PNES development will be reviewed. Strategies for the outpatient multidisciplinary treatment will be presented. Results: The prevalence of pediatric PNES as reported from the Epilepsy Monitoring Units vary from 11% to 30%. It is similar in pre-pubertal boys and girls, but becomes significantly higher in adolescent girls. The median delay in PNES diagnosis is about 3.5 years. At the time of the initial PNES diagnosis, 50­95% of children are taking antiepileptic drugs. A history of sexual abuse is commonly reported in older teenage girls and physical abuse is more frequently identified in younger children. Comorbid epilepsy is more frequently present in children younger that 5 years (48%) as compared to children 5­12 years old (25%), and adolescents (19%). The most common psychiatric diagnoses identified in youth with PNES are Conversion Disorder (77%), Attention Deficit Hyperactivity Disorder (64%), and Major Depressive Disorder (18%). Predictors of positive PNES outcome are younger age at onset, female gender, and multiple "seizure" types. Conclusions: Standardized and population based research studies are needed to generate evidence based clinical data for standardized PNES diagnostic and treatment practice guidelines.

SHOULD WE TREAT EPILEPSY?

Jurgis Strautmanis

Childrens Clinical University Hospital, Riga, Latvia

Background. There is a popular opinion among Latvian parents of epilepsy patients that seizures are rather beneficial than harmful, but anticonvulsants are noxious and should be avoided. This theory comes from rehabilitation center of Philadelphia's "Institutes for the achievement of human potential" (IAHP). Methods. Search of the sources of this theory and counterarguments in the literature. Results. The theory that seizures are beneficial originates in publication of Temple Fay (1942). He raised hypothesis that seizures have been developed during evolution as reflex reaction to hypoxia with aim to help early amphibians get back in water if their abilities to get oxygen in land would appear insufficient. It's based on observations that sei zures occur throughout animal world, can be induced in any brain and ictal movements are typical to amphibians (simple flexor ­ extensor type, never complex). Therefore he considered sei-

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

DIAGNOSIS AND FEEDBACK OF PEDIATRIC NON-EPILEPTIC SEIZURES (PNES) DO'S AND DON'TS

Rochelle Caplan

UCLA, Los Angeles, USA

PREVALENCE AND CURRENT TREATMENT OF ATTENTION DEFICIT ­ HYPERACTIVITY DISORDER (ADHD) IN LITHUANIA PRIMARY SCHOOL PUPILS

Darius Leskauskas

Kaunas University of Medicine, Kaunas, Lithuania

Objectives: This presentation will describe diagnostic assessment techniques that identify psychopathology in children and adolescents with PNES. It will also present clinical guidelines for the effective presentation of a PNES diagnosis to these children and their families. The importance of making a timely PNES diagnosis will be discussed. Methods: A clinical discussion will walk participants through the diagnostic process from when children with PNES are first seen by the neurologist until completion of the assessment of PNES. Brief presentation of clinical cases, in which diagnosis and feedback were successfully and unsuccessfully managed, will support recommendations on "do's and don'ts" when diagnosing children with PNES. Results: Youth with PNES are often undiagnosed and the underlying emotional causes of their condition go untreated. Therefore, PNES has marked morbidity with repeated ER and physician visits, school absenteeism, missed days of work for parents, and unnecessary use of high doses of multiple antiepileptic drugs. This presentation will alert pediatric neurologists to the importance of including PNES in their differential diagnosis of all youth with epilepsy. It will also provide techniques to help pediatric neurologists obtain valid clinical information from these patients and their families, as well as develop new skills for timely diagnosis of PNES. Conclusion: With this information, pediatric neurologists will feel better equipped to diagnose PNES and provide feedback to these children and their families.

ADHD: ALTERATIONS OF DOPAMINERGIC SYSTEM

Aurelija Jucaite

Karolinska Institute, Stockholm, Sweden

The goal of a study was to establish the prevalence of attention deficit ­ hyperactivity disorder (ADHD) in the primary school children in Lithuania and to evaluate what treatment is currently provided for them. Parents and teachers of all pupils of 12 randomly selected primary schools (N=2942) completed the scales on the presents of ADHD symptoms in their children. ADHD was diagnosed if at least 6 symptoms of hyperactivity and/or 6 symptoms of inattention were present for at least 6 months according reports of parents and the result of CAP scale completed by teachers exceeded the norms for age and gender. Results of the both scales were ob tained for 2298 pu pils ­ 1096 girls and 1202 boys 6­12 years of age. ADHD was diagnosed for 5.2% of primary school pupils. It was 1.9 times more frequent among boys than among girls. There was no significant difference between the four grades in the incidence of ADHD in general but there was a relationship between the age of the pupils and the prevalent type of ADHD. Statistically significant relationship was established between ADHD and worse achievements in learning, problems in communications with peers and parents, oppositional defiant behavior. 51% percent of the parents of ADHD diagnosed children have had applied to the mental health care professionals, most of them evaluated the help they received as just partly useful (57.1%) or not useful at all (24.5%). Most of those children had received one consultation of a specialist (17.3%), were offered just psy cho ther apy / be hav ioral ther apy (16.3%) or just pharmacotherapy (8.1%). Only 5.1% of them had received complex treatment.

COGNITIVE DYSFUNCTION OF CHILDREN WITH SYMTOMATIC AND NEWLY DIAGNOSED EPILEPSY

Anneli Kolk

Children's Clinic of Tartu University Hospital, Tartu, Estonia

The hypothesis that altered dopamine transmission underlies hyperactive-inattentive behaviour in children with AttentionDeficit/Hyperactivity Disorder (ADHD) is based on genetic studies, efficacy of psychostimulants, animal models of hyperactivity. Molecular imaging studies, investigating central dopamine system in humans in vivo, have focused so far on binding of dopamine markers in the basal ganglia, midbrain. We investigated dopamine D1 receptor (D1R) density in ADHD children using positron emission tomography and radioligand [11C]SCH23390. Thirteen children with ADHD and twelve comparison children, 9 to 17 years of age participated in the study. Attention and motor behaviour were investigated with a continuous performance task (CPT) and motion measurements, reaction time, visuo-spatial working memory, Stroop test were included in the neurocognitive test battery. The [11C]SCH23390 binding to D1R was decreasing with age in both groups, with highly significant decline in ortical regions in the group of children with ADHD, suggesting intensive and widespread biochemical changes in cortical networks during adolescence.

Background: The aim of this study was to specify the neuropsychological outcome in case of children with the unilateral nonprogressive brain lesion. Method: In order to assess these specific functions, we used a comprehensive model of neonatal stroke with focal epilepsy and newly diagnosed focal epilepsy. The neuropsychological examination was performed using the NEPSY test battery on 44 children aged from 4 to 12 years. The children were divided into three groups: 18 children suffering from neonatal stroke and focal epilepsy, 12 children with newly diagnosed focal epilepsy prior to antiepileptic treatment, and 14 healthy controls matched by sex, age, and socio-economic status. Results: Children with neonatal stroke and epilepsy had more severe cognitive deficit, especially in language, visuoperceptual, memory and learning tasks than children with newly diagnosed partial epilepsy. However, the profile of the cognitive weakness appears to be diffuse and quite similar with both groups. The children with newly diagnosed epilepsy did not demonstrate a clear effect of lateralization, according to the side of epileptic EEG discharges. Especially interesting was the fact that the newly diagnosed epilepsy group showed impairment not only in attention, visuoperceptual and short-

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

term memory skills, but also in auditory perception, lexical function, and the comprehension of speech. Conclusions: It is highly recommended that the children with epilepsy would undergo a neuropsychological examination in order to assess their later cognitive development. consensus defines PS `as a benign age-related focal seizure disorder occurring in early and mid-childhood. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms, and by an EEG that shows shifting and/or multiple foci, often with occipital predominance.' PS probably affects 13% of children aged 3­6 years who have had one or more afebrile seizures and Gastaut-Type 2­7% of the benign childhood focal seizures. Panayiotopoulos syndrome occurs in children who are otherwise normal and is not associated with the development of significant neurodevelopmental problems. Autonomic epileptic seizures and autonomic status epilepticus are the cardinal manifestations. Characteristically the child, who was initially fully conscious, becomes confused, unresponsive and the eyes turn to one side or gaze widely open. Half of the seizures in PS last for more than 30 minutes, thus constituting autonomic status epilepticus, which is the commoner non-convulsive status epilepticus in normal children. Two thirds of seizures occur during sleep, particularly the early part of sleep. Gastaut-type idiopathic childhood occipital epilepsy has entirely different clinical manifestations, despite common interictal EEG features when occipital paroxysms occur in PS. Visual hallucinations, elementary or complex, are the prominent seizure type in Gastaut type and the ictal EEG, when performed is also markedly different. Visual seizures are predominantly diurnal and occur at any time of the day Prognosis in PS is remarkably benign in terms of seizure frequency and evolution while in Gastaut-Type the prognosis is unclear.

22 June, Friday

SEIZURE SEMIOLOGY AND SYNDROMIC APPROAH IN CHILDREN UNDER 3 YEARS

Perrine Plouin

Hopital Necker Enfants Malades, Paris, France

In the classification of epilepsy syndromes accepted in 1989, 5 syndromes were recognized in infancy, none of them with focal seizures. In 2001 eleven syndromes were proposed, and the 6 new ones were concerned with focal seizures. First step is to classify seizures, second to recognize syndromes with clinical, EEG, neuroradiological and genetic data. This improvement in our knowledge on the semiology of seizures in infancy is due to development of longterm EEG video monitoring. Simultaneous polygraphy helps in classification of myoclonic, atonic or tonic components of seizures as well as spasms. In infancy epileptic spasms are the most frequent seizure type; around 1980 were reported focal seizures associated with spasms and asymmetrical spasms; both situations are linked with symptomatic cases although symmetrical spasms are associated with idiopathic or cryptogenic West syndrome. Myoclonic seizures are present in several epilepsy syndromes among neonates and infants. They may be massive, segmentary or erratic. Some of these syndromes are severe such as Early Myoclonic Encephalopathy occurring in neonates, sometimes associated with metabolic diseases. In Benign Myoclonic Epilepsy of Infancy (reflex or not) which belong to IGE, myoclonic seizures are massive as well as in Dravet syndrome or Doose syndrome both cryptogenic. Myoclonus status epilepticus is characterized by erratic myoclonusas in Angelman syndrome but also in progressive neurological diseases as ceroidolipofushinosis. Focal seizures have been precisely analyzed according to the localization and one can easily recognize a frontal, from a central, temporal or occipital seizure in this population where subjective signs are not reported. Benign familial and non familial syndromes with focal seizures should be differenciate from symptomatic focal epilepsies and from epileptic encephalopathies comprising only focal seizures such as MPSI. Recognizing the type of seizures in syndromes in infants is an important challenge considering the etiology, the prognosis and the treatment.

PSYCHIATRIC AND COGNITIVE COMORBIDITIES IN PEDIATRIC EPILEPSY: CAUSE OR EFFECT?

Rochelle Caplan

UCLA, Los Angeles, USA

PANAYIOTOPOULOS SYNDROME VS GASTAUT-TYPE OCCIPITAL EPILEPSY

Anthanasios Covanis

The Childrens Hospital "Agia Sophia", Athens, Greece

Numerous studies demonstrate high rates of a wide range of psychopathology and subtle to marked cognitive difficulties in children and adolescents with both new onset and chronic epilepsy. Despite consistent evidence for a relationship between seizures variables and the cognitive comorbidities of pediatric epilepsy, findings are inconsistent regarding their relationship with psychopathology. This presentation will review studies conducted during the past decade demonstrating the comborbidity of behavioral, cognitive, and linguistic deficits in children with epilepsy. It will discuss the types of epilepsy and/or epilepsy syndromes that are related to specific types of psychopathology and patterns of cognitive and linguistic deficits in these children. Using a developmental model, it will also focus on studies that have examined the role played by seizure related factors and underlying neuropathology in these comorbidities. After a brief discussion of methodological issues in the reviewed studies, it will present a model of epilepsy as a developmental neuropsychiatric illness that presents with different types of seizures, varying seizure control, as well as a wide range of behavioral disturbances and cognitive deficits.

The ILAEs 2001 Diagnostic Scheme recognised two childhood occipital epilepsies, including early onset type benign childhood occipital epilepsy and late onset childhood occipital epilepsy. However, during the last decade independent studies have suggested, that these conditions are likely to be separate disorders, rather than variants of a single disorder and that whilst seizures in Gastaut type occipital epilepsy are indeed likely to originate in the occipital lobes, this is unlikely to be the case in Panayiotopoulos syndrome(PS). An expert

PHARMACOLOGICAL TREATMENT OF ATTENTION DEFICIT HYPERACTIVITY DISORDER IN CHILDREN WITH EPILEPSY

Sigita Plioplys

Children's Memorial Hospital, Chicago, USA

Objective: To review current pharmacological treatment strategies for children with Attention Deficit Hyperactivity Disorder (ADHD) and epilepsy. Background: ADHD negatively affects a child's academic achievement, behavioral and emotional

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functioning, and social interactions. Medications, in addition to educational, behavioral and family interventions, are effective ADHD treatment options. Methods: Research data and clinical information on pharmacological treatment strategies of ADHD in children with epilepsy is presented. Results: ADHD symptoms are reported in 37% of elementary school age children and 25% of adolescents with epilepsy. It is important to establish if there is a temporal association between the development of ADHD symptoms and the course and treatment of epilepsy. It will allow clinician to determine if inattentiveness, hyperactivity and impulsivity are independent developmental symptoms, i.e. ADHD, or represent epilepsyassociated phenomena, such as new onset absence seizures, unrecognized nonconvulsive seizures, or side effects of antiepileptic medications. Further treatment strategies should be determined by this association. Controlled data are available only on the efficacy and safety of methylphenidate (MPH) for the use in children with epilepsy. No published data on the seizure risk are available for Atomoxetine, Guanfacine or Clonidine. Due to the risk of causing or worsening seizures at moderate to high doses, Bupropion is not recommended for children with epilepsy. Conclusions: MPH is effective and safe medication for the treatment of ADHD in children with well controlled epilepsy.

CEREBELLAR ATROPHIES

Eugen Boltshauser

University Children's Hospital, Zurich, Switzerland

CURRENT ACHIEVEMENTS IN THE GENETICS OF THE EPILEPSIES

Ulrich Stephani, Hiltrud Muhle, Sarah von Spiczak

Pediatric University Hospital, Kiel, Germany

An imbalance of excitatory and inhibitory mechanisms in the brain is postulated to be the global pathogenic mechanism in epilepsies. Since the electrical excitability of membranes mainly is performed by ions and neurotransmitters it was not surprising that mutations of ion channels and neurotransmitter receptors recently have been discovered to be associated with monogenic idiopathic generalised and focal epilepsies (IGE, IFE): Mutations of channels for calcium ions with childhood absence epilepsies, for potassium ions with benign neonatal familial convulsions, for sodium ions in generalised epilepsies with febrile seizures plus, severe myoclonic epilepsy of infancy and others, for chloride ions with different forms of idiopathic generalised epilepsies, for acetylcholine receptor subunits with a certain fontal epilepsy, for GABA-receptors with different forms of IGE. Only the mutated genes for the protein myoclonin1 in juvenile myoclonic epilepsy and for the leucine rich glioma inactivated protein 1 in lateral temporal epilepsy do not belong to this group. Despite the success of discovering these pathogenic mechanisms in the majority of epilepsies the respective mutations or even polymorphisms could not be found yet. In this context it is noteworthy that common complex diseases such as epilepsy do not follow classic Mendelian inheritance patterns and it is unlikely that epilepsy is caused by single gene defects. One of several different hypotheses suggests that the disease-predisposing alleles occur frequently in the population and each of them contributes little to disease susceptibility (known as the common variant, common disease ­ CVCD ­ hypothesis acc. to Collins et al. 1997). In order to advance the understanding of pathogenetic mechanisms in epilepsy it seems to be necessary to further endophenotype the epileptic syndromes e.g. by EEG marker being associated with them. Ongoing research on the photoparoxysmal response, its clinical association with the epilepsies and the molecular genetic analyses will be shown.

The term Cerebellar Atrophy (CA) implies neuroimaging evidence of secondary loss of cerebellar tissue. CA may be seen as a residual change resulting from a single "hit" (e.g. an intoxication), or it may represent an ongoing process in the context of a progressive disorder. CA may be global, or more localised, involving either mainly the vermis, or affecting predominantly the hemispheres. CA can be accompanied by brainstem / pontine atrophy. Depending from the underlying disease supratentorial atrophy can be present. Differentiation between hypoplasia, prenatal onset atrophy and even postnatal onset atrophy is not always possible on a single neuroimaging study. It is well documented that congenital non-progressive ataxias may appear on MRI like CA, with dilated / enlarged forth ventricle and enlarged interfoliar spaces. To complicate the situation further, CA can be superimposed on cerebellar hypoplasia, as seen in some patients with congential disorders of glycosylation. It is important to recognise that not all progressive ataxias have CA on neuroimaging (e.g. Friedreich ataxia, Refum disease), while CA may be demonstrated in patients in whom progressive cerebellar dysfunction cannot be demonstrated in view of their neurological imparment (e.g. PEHO syndrome). In the pediatric age acquired CA are less prevalent than genetic CA, but can occur in a large spectrum of conditions, including post-inflammatory, post-traumatic, post-asphyctic, toxic, paraneoplastic, nutritional (vi tamin B12 de fi ciency), and fol low ing ex treme prematurity. CA is a feature of a large group of inherited progressive disorders (isolated, or with cerebral atrophy, or with white matter involvement). The MRI appeareance of "genetic" CA is mostly nonspecific. Exceptions are the cerebellar cortex hyperintensities seen in infantile neuroaxonal dystrophy and Marinesco-Sjögren syndrome. The diagnostic approach should be based on the individual constellation based on history, age, clinical features, and metabolic screening tests.

GENETICS OF MENTAL RETARDATION: PRESENT AND FUTURE

Lina Basel-Vanagaite

Schneider Children's Medical Center of Israel, Petah Tikva, Israel

Mental retardation (MR) is a highly heterogeneous condition with a prevalence of 1­3% in the general population. The psychosocial burden on families with mentally handicapped children is extensive. Genetic etiologies are found in approximately two thirds of mental retardation cases. MR affects 30­50% more males than females, and X-linked MR is thought to account for this sex bias. It can be subdivided into syndromic forms, which are characterized by MR and either malformations, dysmorphic features, or neurological abnormalities, and nonsyndromic forms, which are characterized by MR without any additional manifestations. Etiological diagnosis and genetic counseling for MR is one of the most difficult challenges faced today by clinical geneticists. Only a few recurrent genetic defects exist, such as Down syndrome and Fragile X syndrome. For nonsyndromic MR, more than 80 loci are described and 28 genes have currently been identified. For syndromic MR, more than 290 genes have been identified. Abnormalities in DNA copy number are frequently found in patients with multiple congenital anomalies/MR syndromes and can be detected by array-based comparative genomic hybridization (array-CGH). We present an overview of recent achievements in the elucidation of the genetic defects causing MR. A comprehensive strategy to systematically identify

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the causative genetic defects in patients with MR using a combination of routinely used and recently developed laboratory tests is described. Some examples of prevention of MR in specific populations are given. and/or deprivation of an indispensable product. In the neonatal period neurometabolic disorders may present with seizures, floppiness, impaired consciousness, feeding problems, hypothermia, facial dysmorphisms, micro- or macrocephaly or hydrops fetalis. In the early infantile life failure to thrive, poor head control, recurrent infections, intractable convulsions, hyperventilation (central hyperpnoea), skeletal deformities and eye problems (impairment of vision, corneal clouding, nystagmus etc.) are additional symptoms. Often there is a combination of symptoms. The nervous system is more often involved in IMD than any other organ because the immature brain in the newborn is most susceptible to biochemical derangement. There are about 100 IMD which may come to clinical expression in the first weeks of life. This group of metabolic diseases comprise genetic defects in the metabolism of amino acids, neurotransmitters, fatty acids, lipids and cho les terol, car bo hy drates, pur ines and py rimi dines, creatine, and glycoproteins as well as mitochondrial respiratory chain disorders, urea cycle defects, lysosomal storage diseases and peroxisomal diseases. Scarcely 20 are amenable to treatment. The presentation of metabolic diseases is often characterized by life-threatening decompensation demanding prompt and resolute action. First of all adequate samples must be obtained for basic laboratory investigations: blood gases and plasma electrolytes, plasma glucose, lactate and ammonia, and urinary keton bodies to cover differential diagnostics. Results of those analyses should be available within 30 minutes, at the latest. In addition adequate samples should be taken for spe cial met a bolic in ves ti ga tion: acylcarnitines (dried blood spots), plasma amino acids, urinary organic acids. Results of special metabolic investigations, relevant to the diagnosis of potentially treatable metabolic disorders should be available at the lat est within 48 hours [1]. The aim of this presentation is to emphasize the importance of inherited metabolic disorders to become included in the differential diagnostics of neurologic diseases.

FUTURE TREATMENTS OF NEUROMUSCULAR DISORDERS

Thomas Sejersen

Karolinska Institute, Stockholm, Sweden

"What new promising treatments are around the corner", we are often asked and ask ourselves. This question may be viewed from different perspectives. We will look at them from two of these: a time aspect and a pathophysiological aspect. Looking at future treatments from a time perspective there are three major levels, diffusely denoted present time, near future, and distant future. Although dealing with the future we will have to start at the present time. Do we now treat according to the best available knowledge? How do we know if we do so? The task to establish and follow an already present golden standard of treatment is here a first important step. In the near future we have treatments in or close to later phase clinical trials that are likely to result in additions in the therapeutic arsenal. In a more distant future we foresee novel therapies that are now at a state of basic research or early clinical trials, and also not yet defined strategies. Among these are means of directly compensating for the genetic alteration that often underlie neuromuscular disorders. If we instead look at future treatment options from a pathophysiological aspect, we can see areas of treatment and care arising that are directed at 1) counteracting the primary pathogenetic mech a nism un der ly ing the dis or der (e.g. corticosteroid treatment of DMD, medical attempts to increase SMN2 levels in SMA, and gene therapies), or 2) symptomatic for an affected tissue or organ (e.g. ACE-inhibitor treatment of approaching cardiac insufficiency, scoliosis surgery, ventilatory support etc). Yet another level we need see delevoped in the future is a global habilitation/rehabilitation view for measures that are supportive for well-being and life in society, including measures to increase independence, ease communication, and participation in society, e.g. in the educational system and at work. No matter what perspecitve we look at future measures, a key feature is that large efforts are needed at a local plane, but more importantly, at an overall multinational level. Therefore, the best measure to allow for this improvement in future therapies at various levels is the creation of large international networks, e.g. TREAT-NMD, to implement the best current knowledge, to increase awareness in society, and to collaborate for finding novel theraputic strategies, and to allow for continuously ongoing, good clinical trials to improve present therapies and test new ones.

ORPHAN DRUGS AND RARE DISEASES: PROBLEMS AND OPPORTUNITIES

Arrigo Schieppati

Mario Negri Institute for Pharmacologica Research, Ranica, Italy

INHERITED METABOLIC CAUSES OF NEUROLOGIC DISEASES IN NEONATES AND INFANTS

Leo Spaapen

Academic Hospital Maastricht, Maastricht, The Netherlands

Neurologic disturbances are major characteristics of many well-known inherited metabolic diseases (IMD). IMD are caused by mutations in genes resulting in a structurally altered inactive protein, often an enzyme. The enzyme defect may cause a block in a metabolic pathway, resulting in accumulation of a metabolic substrate which may be neurotoxic

There are 4 to 5 thousand different diseases that affect a very limited proportion of patients. Most of these diseases lack an appropriate treatment, for several reasons. One reason is the limited knowledge of pathogenesis and pathophysiology, which is in turn related to the lack of animal models and to the difficulty of collecting enough patients to obtain a significant number of observations. However, there is another important aspect to be considered: rare diseases occur so infrequently that most of the times there is no reasonable expectation that the cost of developing and making available a drug for the treatment of such diseases will be covered by sales. Patients with rare diseases have been named the "Health Orphans" and the treatments that could cure them `Orphan Drug'. Orphan Drug legislations in US offer incentives consistingn in market exclusivity for orphan designated products with Food and Drug Administration approval, and research tax credits for rare disease clinical studies. Before 1983 only 7 drugs were available to treat rare disease. Since then, more than 1600 drugs and biologics have been designated as orphan products and more than 200 of these have received FDA marketing approval. The European Union after lengthy proceedings and delays has finally approved a Regulation for Orphan Medicinal Products. The European regulation is applicable to disorders with a prevalence of no more than 5 per 10,000, or for a life-threatening or seriously debilitating communicable disease even when the prevalence is higher. The European regulation, although has been approved after a long awaiting,

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has nonetheless raised few criticisms from the industry and from the academia. These criticisms notwithstanding, patients with rare diseases have now much more opportunity for treatment than they before in America and in Europe, although many problems are still to be solved. unmet medical needs of patients suffering from rare diseases within Community, as they deserve access to the same quality of treatments as other patients. This legislation is part of a broader Community policy to identify rare diseases as a priority area for action in the field of public health. Member States must adopt specific measures to increase knowledge on rare diseases and to improve their detection, diagnosis and treatment. Lithuanian measures concerning rare diseases and conditions and national incentives for orphan medicinal products are insufficient. Firstly, the definition of `rare disease or condition' laid down in the Decree of the Health Ministry State Patient Fund Directorate do not meet the EU standards. Secondly, there are no statistical data on the number of patients suffering from rare diseases or conditions in Lithuania. The other problems: there are no specific patients' organizations for patients suffering from rare diseases and their families. There is a lack of information on rare diseases not only for patients, but also for the health care professionals. These problems are in insignificant part and temporarily solved through patients' participation in clinical trials. The Lithuanian Government promotes the use of orphan medicinal products for specific patients by reimbursement from the funds earmarked for that purpose in the budget of the compulsory health insurance fund (which is only 5 mll. LTL in 2007). Individuals are compensated for medicinal products for rare diseases and conditions on presenting specialist doctors' reports, following a decision by the Committee approved by the Health Ministry State Patient Fund responsible for taking decisions regarding treatment of rare diseases and conditions.

ACCESSING INFORMATION ON ORPHAN DRUGS: THE EUORPHAN SERVICE

Paola Baiardi 1, Manuel Posada de La Paz 2, Lars Savendahl 3, Domenica Taruscio 4, Renza Barbon Galluppi 5, Enrico Morten 6, Donatas Stakisaitis 7, Adriana Ceci 8

1 2

Consorzio per Valutazioni Biologiche e Farmacologiche, Pavia, Italy; Foundation for Cooperation and International Health Carlos III, Spain; 3 Karolinska Institutet, Stockholm, Sweden; 4 Istituto Superiore di Sanita, Italy; 5 UNIAMO, Federazione Italiana Malattie Rare, Italy; 6 Softeco Sismat S.p.A., Italy; 7 Vilnius University, Vilnius, Lithuania; 8 Consorzio per Valutazioni Biologiche e Farmacologiche, Italy

Approximately 30 million people currently suffer from rare diseases in Eu rope. De spite the reg u la tory in ter ven tions (141/2000/EC Regulation) to propel the development of drugs for the care of rare disorders (orphan drugs), few treatments are still available and patients find many difficulties in accessing quality healthcare for their disorders. Moreover, whenever available, information on orphan drugs is very limited, fragmentary and difficult to retrieve because of the lack of a unique data source. EuOrphan (www.euorphan.com) is a service developed under the auspices of the EU eTEN Programme aimed at distributing a complete set of information on orphan drugs commercialised or still under development (designated drugs) worldwide. The service addresses all the stakeholders in the field: patients and patient associations, physicians, researchers, public health institutions and pharmaceutical companies. It provides four main services: · the access to a centralized database containing standardised information on about 1500 designated and marketed orphan medicines. Availability throughout Europe, prices and usage in paediatrics are the added value information included in the database; · statistics on the orphan market at a worldwide level supporting public health authorities in planning a rationale usage of resources and public health interventions; · a consultancy service managed by a team of qualified experts supporting pharmaceutical companies in developing new orphan drugs and complying with regulatory requirements; · a forum constituting a useful means to facilitate contacts among the different actors, to organize pressure groups and to stimulate new studies. Finally, a news section is updated monthly and newsletters are provided upon registration quarterly. EuOrphan full service has been running since June 2006. It is currently funded by public and private supporters that consider access to high quality information a central means to provide social and ethical benefits to disadvantaged minorities of patients.

PUBLISHING IN BIOMEDICAL JOURNALS: HINTS FOR SUCCESS FROM A JOURNAL EDITOR

Roger Brumback

Creighton University School of Medicine, Omaha, USA

ORPHAN PATIENTS AND ORPHAN MEDICINES: SITUATION IN LITHUANIA

Indre Spokiene

Mykolas Romeris University, Biolaw Department, Vilnius, Lithuania

The EU orphan legislation entered into force in April 2000. Orphan medicinal product legislation was timely to address the

In medical schools, colleges, and universities throughout the world, academic rank, tenure, and recognition are based in large part on scholarly achievement, including publication of case materials and of scientific investigations. Arguments abound whether quality or quantity of publications is more important (wags have even suggested that "promotion committees can count but not read"). Nonetheless, it is necessary to understand the process of journal article preparation and submission to survive the gauntlet of editorial evaluation and peer review in order to achieve publication. The initial step should be the appropriate formulation of the question to be answered in the article. It is imperative to demonstrate the importance of this question in order to justify the addition of this article to the world's published biomedical literature. Preparation of the manuscript of the article must follow not only all the detailed instructions for authors, but also representative published examples from the particular journal to which the manuscript will be submitted. The first part of the article to be written is the case summaries and/or methods section which needs to be comprehensive. The results and discussion sections should then follow, showing what is unique in this article and stressing why it is worthy of publication. Figures and tables should be prepared in a manner to stand alone, providing sufficient information that reading the article text is not required for understanding. Computer-generated figures must have sufficient detail and be of high enough quality to be clearly visible when printed in the journal. The completed manuscript for submission should be as close to perfect as possible, since the submitted material will constitute the journal editor's first impression of the quality of the author's scholarship.

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23 June, Saturday

EPILEPSY, MAGNESIUM, GENDER DIFFERENCES

Jurgita Grikiniene

Vilnius University Children's Hospital, Vilnius, Lithuania

Background: Recent research has found gender differences in prevalence and clinical manifestation of epilepsy in humans, in susceptibility to proconvulsants in animals, genderrelated differences of Mg, Na+ and Cl­ metabolism, adverse drug effects that depend on the gender of patients, but certain mechanisms of these effects are not clear. Magnesium (Mg) is involved in the mechanisms of neuronal susceptibility to excitability, including epilepsy. Mg2+ inhibits the facilitating effect of calcium on synaptic transmission and also exerts a voltage- de pend ent block age of the N-methyl-D-aspartate (NMDA) receptor. It is well known that low Mg2+ levels induce spontaneous epileptiform activity in several cortical structures. Several studies suggest that the body electrolytes and some trace elements may be causally involved in some forms of epilepsy and also to increase the recurrence of seizures. Mg urinary excretion in epileptic patients have not been investigated earlier. The aim of the study: to define peculiarities of urinary Mg excretion in epileptic adolescents without treatment (newly diagnosed epilepsy) and to elucidate gender-related differences. Methods: We studied 52 untreated epileptic children (newly diagnosed epilepsy): 23 boys and 29 girls, and 95 control children (47 boys and 48 girls) aged 11­17 years. Epileptic children were treated in Department of Children Neurology, Vilnius University Children's Hospital. The clinical diagnosis was made according to the criteria of the International League against Epilepsy (1981). All studied children did not suffer from another acute or chronic disease. 24-h urine was collected. Urinary Mg levels were measured with a spectrophotometer. Urinary pH and creatinine levels were measured also. Results: 24-h urinary Mg excretion of epileptic girls was significantly higher compared with control girls. These differences were not found in untreated epileptic boys. Conclusion: Our data showed altered urinary Mg excretion in epileptic adolescent girls.

1­5 years: in 5 idiopathic generalised; in 7 partial idiopathic or criptogenic extratemporal and in 2 temporal; in 3 symptomatic par tial. Af ter FS two chil dren had ep i lep tic encephalopathy; 2 had syndromes with molecular abnormalities; 1 had Fahr disease. One girl presented for the first time with febrile status epilepticus had Sturge Weber syndrome. Between 23 children with epilepsy, previously 13 had one and 10 had complex FS. Betwen 23 children with epilepsy, good control of epilepsy had 7 children; normal intellectual development had 6 children. Conclusion: Reviewing our 880 patients with FS, 2.6% developed epilepsy. Between them 2 children had refractory idiopathic temporal lobe epilepsy. Hippocampal sclerosis was not found in our children with FK and subsequent epilepsy.

SEIZURES AND VALUE OF EEG INVESTIGATIONS IN ASPHYXIATED TERM NEWBORN INFANTS

Ulvi Vaher, Aita Napa, Tiina Talvik

Children's Clinic of Tartu University Hospital, Tartu, Estonia

FEBRILE SEIZURES AND EPILEPSY: A RETROSPECTIVE STUDY

Ljerka Cvitanovic Sojat 1, Masa Malenica 1, Romana Gjergja 1, Zlatko Sabol 2, Tina Sojat 1

1 2

UH Sestre milosrdnice, Zagreb, Croatia; Pediatric Outpatient Clinic `Dr. Sabol', Croatia

Background: Febrile seizures (FS) precede an onset of various forms of epilepsies in 10­15% of children. Risk for epilepsy in children with FS is 3% by the age of 7 years. Complex FS are associated with a younger age at onset of epilepsy. One-third of patients with temporal lobe epilepsy have a previous history of prolonged febrile seizures. The aim of this study was to determine types of FS, recurrences and occurrence of epilepsy in our patients with FS. Method: Retrospective analysis of data of patients with FS treated at our Neuropediatric Unit and Outpatient Clinic last 20 years was done. Results: 880 patients had FS: simple 81.1% and complex 19.8%. Thirty-three children had subsequent non-febrile seizures. Epilepsy occurred in 23 children following FS after

Neonatal seizures are markers for perinatal brain damage and has been correlated with later neurological sequelae. Birth asphyxia is a major cause of perinatally acquired brain injury in full-term infants. A characteristic feature of neonatal seizures is the phenomena of electroclinical dissociation: seizures can be electro-clinical, electrographic (subclinical) or clinical only. The incidence of seizures in infants born at term is 1.5­3.0 per 1000 live births. The incidence of electrographic seizures is unknown. There is now evidence that electrographic-only seizures have a similar impact long-term outcome as electroclinical seizures. The prognostic value of electroencephalography (EEG) in full-term infants with hypoxic ischemic encephalopathy (HIE) has been well documented. The aim of the study was to evaluate the presence of neonatal seizures and EEG background activity in asphyxiated term infants. Patients and methods. The study group included 22 term asphyxiated neonates admitted to Children's Clinic of Tartu University Hospital (Children's Intensive Care Department and Department of Neonatology). 17/22 were born in severe and 5/22 in moderate asphyxia. In 18/22 of them developed HIE (8/18 mild, 7/18 moderate, 3/18 severe). 9/22 were diagnosed clinical features of neonatal seizures. First EEG was recorded on the 1.­4. day of life. Electroclinical association between clinical seizures and EEG findings was established in 6/9. 3/9 newborns had normal EEG features (recording was done at day 2­4). In 3/22 neonates without clinical seizures 2 babies had electrographic seizures and one baby had moderate abnormalities in background activity. EEG in these babies was done in 5 day of life 2/3 and at the first day of life 1/3. Conclusions: 1) in all neonates born in asphyxia EEG recording is needed for evaluation of seizures and background activity; 2) the EEG recording should be done as early as the first day of life; 3) for prognostic purposes serial recordings are needed.

NEURODEVELOPMENTAL OUTCOME OF NEONATAL SEIZURES

Jurate Grigoniene, Dovile Rackauskaite, Milda Endziniene, Nerija Vaiciene

Kaunas University of Medicine, Kaunas, Lithuania

Objective. To determine neurological outcome of neonatal seizures and factors that may influence it. Methods. We have retrospectively analyzed 76 cases of neonatal seizures at the Department of Neonatology of Kaunas

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Medical University Hospital within 2000­2003. As the second step questionnaires were sent by post to the parents of children who have experienced seizures in the neonatal period. Results. Response rate was 47%. Twenty children (55%) of 2­5 years old had normal development and no seizures, 2 children have died. Fourteen children (39%) had abnormal development: 9 (25%) had cerebral palsy, 4 ­ language disorder, 1 ­ mixed developmental disorder. Seven children (19%) had epilepsy and were on antiepileptic drugs, four of them had also cerebral palsy, one ­ language disorder. Even six children with epilepsy had abnormalities on ultrasonography (US), performed during neonatal period. There was no significant difference in the neonatal neurological status between children who later developed seizures and those who did not. Only 6 children had electroencephalography (EEG) performed in neonatal period, so the information for the analysis was insufficient. All children with developmental disorders had abnormalities on US in neonatal period; 36% children with normal development had changes in US (p<0.01). Twenty-six children (74.3%) experienced seizures during the first two days of life, and impaired development or cerebral palsy were more common in this group to compare with those who experienced seizures later (p<0.05). Conclusions. Seizures during the first days of life were related to impaired neurological development. Neonates who experienced seizures but had no abnormalities on US had more favourable outcome. EEG would be helpful to predict prognosis of neonatal seizures.

PHYSICAL CHARACTERISTICS OF UNAFFECTED LIMBS OF CEREBRAL PALSIED CHILDREN

Naile Guney 1, Ugur Cavlak 2

1

Physical Therapy Practioner at a Private Rehab. Center, Denizli, Turkey; 2 Pamukkale University, Turkey

Background: The purpose of this study is to evaluate physical characteristics and functional level of the unaffected side of the hemiplegic cerebral palsied (HCP) children and to compare with healthy subjects. Method: A total of twenty-five children with HCP aged 6 and 12 years (mean age; 9.16±1.92 years) who were attending local rehabilitation centers in Denizli and Burdur were included in the study and were compared with those of matched 25 healthy children among the same age group. Before the testing; the demographics belonging to the sample such as age, gender, weight, height and antropometric measurements (AM) were recorded. Body mass index score, grip strength, skinfold caliper measurements (SCM), upper extremity (M.Biceps Brachii, M.Triceps Brachii) and lower extremity (M.Quadriceps Femoris, Hamsring Muscles) muscles strenght were examined. To measure functional level of the subjects; balance tests (eyes open and closed, one leg standing), half squat performance and one leg-heel raise test were used. Results: No statistical significant differences regarding AM were found between the groups. (p=0.05). Extremity disparency measurements, except for tight, also showed no significant difference between both groups (p=0.05). The same results were observed for SCM (p=0.05). While there were no significant differences regarding upper and lower extremities muscles strengths between both groups (p=0.05), a significant difference regarding handgrip strength score in favor of healthy subjects was found (p=0.05). When the balance, half squat and one leg-heel raise tests score were analyzed, the results showed that the children with HCP had lower scores in all functional tests than those of matched healthy subjects (p=0.05). Conclusion: The results of this study show even though the children with HCP had the same physical characteristics in unaffected side like healthy subjects, they had lower functional level than that of matched healthy children. Thus, physical therapists should evaluate both physical characteristics and functional level of the children with HCP .

TREATMENT OF SPASTICITY WITH BOTULINUM TOXIN A IN CEREBRAL PALSY: THREE YEARS EXPERIENCE AT KAUNAS UNIVERSITY HOSPITAL

Svajune Gradeckiene 1, Danguole Ostrauskiene 1, Lina Norkiene 2, Asta Kutraite 1, Milda Endziniene 1, Nerija Vaiciene 1

1

Department of Neurology, Kaunas University of Medicine, Kaunas, Lithuania; 2 Department of Rehabilitation, Kaunas University Hospital, Kaunas, Lithuania.

Background: Treatment of children with cerebral palsy with Botulinum toxin A was introduced at our department in 2004. The aim of the study was to evaluate the efficacy of the treatment on the spasticity of the lower limbs. Method: We have analysed data of 49 children with cerebral palsy between 2 and 9 years of age. The scores of spasticity (Ashwort scale), gait cycle analysis, foot dorsiflexion and the level of Gross Motor Function Classification System (GMFCS) were assessed before injection and one month after each injection. The number of injections varied from 1 to 6 for the same patient. Injections were performed in gastrocnemius, soleus and adductor muscles. Statistical analysis was performed using SPSS program package, significance assumed at p < 0.05. Results: After Botulinum toxin A injections 59.4% of the patients have reached higher functional level according to GMFCS. It was more significant in patients who had lower baseline spasticity. Visits and injections at regular intervals as well as cast wearing significantly improved GMFCS functional level and gait to compare with irregular ones. Higher number of injections was related with increased dorsiflexion of foot. Conclusion: Lower spasticity at baseline, regularity and number of injections were the main factors that influenced more favourable treatment outcome of spasticity in children with cerebral palsy.

MUSCULAR PRESENTATION OF INHERITED DISORDERS OF MITOCHONDRIAL METABOLISM IN CHILDREN

Birute Skerliene, Jura Tuleviciene, Marija Jakutovic, Donatas Petroska

Vilnius University, Vilnius, Lithuania

Muscle pathology is a common presentation of inherited disorders of mitochondrial metabolism (IDMM) ­ respiratory chain deficiencies (RCD) and fatty acid oxidation defects (FAOD). It can manifest as the fatal infantile myopathy with severe progressive and generalized weakness, respiratory distress and lactic acidosis, or as myopathy with hypotonia and progressive weakness of the limbs, intolerance of exertion and muscle atrophy in the cases of RCD. Myalgia or myoglobinuria are occasionally observed in RCD, whereas recurrent acute rhabdomyolysis (AR) is characteristic of FAOD. Pathomorphological alterations are usually helpful to sustain the suspicion of RCD, whereas biochemical investigations of blood and urine are necessary for FAOD diagnostics. Here we present the peculiarities of the muscular presentation in the

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patients with the different IDMM. Patient 1: Kearns Sayre syndrome in 13 years old boy with ptosis, chronic progressive external ophthalmoplegia, muscle hypotrophy, growth retardation, decreased higher mental function, hystochemical alterations of the muscle tissue. Patient 2: myopathy, peripheral neuropathy, intestinal pseudoobstruction, lactic acidosis and pathomorphological alterations of the muscle tissue in 1 month old boy. Patient 3: Barth syndrome in 8 years old boy with 3-methylglutaconic aciduria, myopathy, cardiomyopathy, cyclic neutropenia, episodes of hypoglycemia, severe Reye syndrome-like episode with coma and subsequent severe neurological deficiency, CT abnormalities. Patient 4: longchain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency in 8 years old girl with the recurrent episodes of metabolic encephalopathy in infancy and recurrent episodes of AR since the age of 3 years with characteristic dynamics of creatine kinase (CK) and other sarcoplasmic enzyme (SE) activity. Patient 5: 12 year old girl with short-chain coenzyme A dehydrogenase deficiency and recurrent episodes of AR with characteristic dynamics of CK and other SE activity. Conclusion: AR was shown to be characteristic of FAOD, chronic myopathy of various severities and peculiar pathology of multiple organs ­ of RCD. velopmental milestones are significantly delayed or never achieved. As the child ages, limb spasticity usually replaces the hypotonia, but the child has poor head control and does not learn to sit unsupported, much less walk. These patients may survive to the sixth decade of life or longer. We report a patient who had severe muscle hypotonia, hyperkinetic hand movements and nystagmus since birth. Three maternal brothers and patient's own brother have died in infancy, they were diagnosed to have had "seizures" or "cerebral palsy". On the 3rd day of the patient's life swallowing difficulties occurred. At the age of 4 months he had his first paroxysm: tonic contraction of facial muscles of one side, head devi a tion to the same side, dystonic posturing of the extermities, blinking of one eye. The baby seemed not to lose his consciousness during these episodes, they most likely were unpleasant as the patient very often he seemed to be unhappy and cried at the moment. These episodes were multiple per day, usually around sleep or when the baby was unhappy (tired, hungry). At the age of 5 years the patient is still hypotrophic and has severe developmental delay: he is no speech, no head control or any voluntary purpuseful movements. He smiles and makes joyful sounds as the reaction but no other verbal or motor contact is possible. Brainstem auditory evoked potentials showed bi lateral impairement of acoustic nerve, absence of 5th wave. Triplication of PLP1 gene has been found which may explain the very severe course in this case.

JUVENILE ONSET HUNTINGTON DISEASE

Nerija Vaiciene

Kaunas University of Medicine, Kaunas, Lithuania

Huntington disease is distinguished by the triad of dominant inheritance, choreoathetosis and dementia. The usual age of onset is in the fourth and fifth decades, but 3­5% of cases begin before the 15th year and some even in early childhood. We report 15 year-old girl who was consulted at our department for the first time at the age of 11 years and clinically presented with unbalanced gait, impaired speech and increasing difficulty with fine motor control since the age of 6 years. Initially myotonic dystrophy was suspected based on the symptoms of weakness of facial muscles (opened mouth, slight ptosis), dysartria, muscle hypotrophy and weakness, low intelligence. Nerve conduction velocity was normal, EMG was impossible to perform because of patient's reluctance. Her mother was healthy; father's family history was unknown. After 3 years her clinical picture has changed, the most prominent symptoms became rigidity and bradykinesia. Imaging studies showed diffuse enlargement of the lateral ventricles. Molecular genetic investigation revealed more than 80 CAG repeats which confirmed the diagnosis of Huntington disease. Conclusion. The clinical features of juvenile Huntington disease are often far from classical, giving rise to diagnostic difficulties. Rigidity, bradykinesia, dysarthria are the most prominent features such that Huntington disease should be suspected in children and adolescents presenting with Parkinson-like syndrome.

MELATONIN SECRETION IN CHILDREN: DEVELOPMENT AND NEUROLOGICAL DISORDERS

Ruta Praninskiene 1, Aurelija Jucaite 2, Irena Dumalakiene 3, Mykolas Mauricas 3

1 2

Vilnius University Children`s Hospital, Vilnius, Lithuania; Karolinska Institute, Stockholm, Sweden; 3 Vilnius University Institute of Immunology, Vilnius, Lithuania

The endogeous circadian rhythm of melatonin (MLT) secretion is generated by the suprachiasmatic nuclei and entrained by the light-dark cycle. Children with neurological and psychiatric disorders, such as lesions of the prefrontal cortex, hypothalamus and disorders of the superior cervical ganglia, blindness, Rett and Angelmann syndromes, cerebral palsy, epilepsy, depression, autism, have a wide variety of pathological changes of the brain which result in fragmentation of sleep. It is hypothesized that altered regulation of endogenous MLT secretion may be an important factor influencing sleep patterns. We investigated diurnal MLT secretion in saliva and excretion of the melatonin metabolite, 6-sulfatoxymelatonin, in typically developing children. Twenty nine children (17 girls, 12 boys), from 5.4 to 17.2 years of age, without sleep disorders, with cephalgia and osteochondrosis, were investiged in the Department of Child Neurology, Vilnius University Children's Hospital. Saliva and urine samples were collected during a 24 ­ hour period every 3 hours. Immunoassay, ELISA kits (Bühlmann Laboratories AG, Switzerland) were used for the MLT measurements. Childrens' sleep was assessed using standardized questionnaire. Results showed high inter-subject variability of MLT concentrations. Mean daytime concentration of 6-sulfatoxymelatonin was related to age (p=0.03). There was no effect of gender, age, endocrinological age on MLT saliva concentrations. Interestingly, peak MLT saliva levels (at 3.00 am) correlated with gestational age (r = 0.36; p = 0.05). Sleep scores had no relation to MLT levels, but also correlated with childs gestational age. Thus, preliminary results show that relation between endogenous MLT levels and sleep patterns is complex and may be influenced by other important factors that determine childs development.

A CASE OF SEVERE PELIZAEUSMERZBACHER DISEASE CAUSED BY THE TRIPLICATION OF PLP1 GENE

Milda Endziniene

Kaunas University of Medicine, Kaunas, Lithuania

Pelizeus-Merzbacher disease is an X-linked neurological disorder. Clinical signs include combination of nystagmus, stridor, hypotonia, spastic quadriparesis, cognitive impairment, ataxia, tremor, and diffuse leukoencephalopathy on MRI scans. Seizures and perinatal stridor are rare signs, typically seen only in the most severe cases. Growth is poor; de-

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

SLEEP DISORDERS IN CHILDREN WITH DEVELOPMENTAL DISABILITIES

Ruta Samaitiene, Laima Mikulenaite, Liuda Istigeceva

Vilnius University Children Hospital Child Development Center, Vilnius, Lithuania

Poster Presentations

PERINATAL NEUROLOGY AND DISABILITY

VARIATION IN LANGUAGE DEVELOPMENT AMONG SENSORINEURAL BILATERAL HEARING IMPAIRDED CHILDREN AFTER FITTING WITH A HEARING AID OR COCHLEAR IMPLANT

Sandra Kuske, Ilze Mezinska

SIA Veselibas centrs "Bikernieki", Riga, Latvia

Objective: To evaluate the clinical symptoms and ethiology of sleep disorders of children with developmental disabilities. Methods: prospective study of a sample of 30 children with developmental disabilities, treated in Child Development Center during 2006­2007. Patients with parental complaints of sleep problems were studied. Several aspects of sleepwake behaviour were analysed using sleep questionnaire, sleep diary, interview regarding child's medical and sociofamilial history, psychological difficulties; video recording of sleep; actigraphy. All patients receive psychological testing. Results: Subject consisted of children with mental retardation, language and learning disorders, pervasive developmental disorders/autism spectrum disorders, cerebral palsy. Age range was 1­6 years. We found a wide range of frequently occurring sleep disturbances in their children. Symptoms of insomnia, parasomnias, and limit-setting sleep disorder featured prominently. Extrinsic sleep disorders (limit-setting sleep disorder, sleep-onset association disorder) featured in younger children with language disorders and disturbed mother-child relationships. Parasomnias and disruptive night time behaviour were main sleep problems in children with pervasive developmen tal disorders/autism spectrum disorders. Sleep related epilepsy was found in 2 cases. Conclusion: Insomnia and parasomnias are very prevalent among sleep disorders in children with developmental disabilities. Multiple child and family factors are associated with sleep disorders.

The number of sensorineural bilateral hearing impairements who need further adeguate medical, technical, paedagogical, social and psychological support increasing. Technical support basically depends on the degree of hearing loss. Cochlear implanted children worldwide demonstrates the established method and adeguate technical supply to open up possibilities for auditory perception and language development for the hearing impaired child. Depending on individual characteristics at the moment of implantation, the performance of children who receive a cochlear implant varies.

SAMPLE OF COMPLEX NEUROREHABILITATION FOR CHILD WITH MULTIPLE DEVELOPMENTAL ANOMALIES

Aija Kaulina, Imants Lanka, Agnese Kretaine, Zane Tumsevica, Inese Stepko

Center of Interdiciplinary Child Development, Liepaja, Latvia

ENDOVASCULAR TREATMENT OF INTRACRANIAL ANEURYSMS IN CHILDREN ­ FIRST EXPERIENCE

Karlis Kupcs 1, Helmuts Kidikas 1, Igors Aksiks 1, Svetlana Rudnicka 2, Aleksejs Vinogradovs 1, Janis Savlovskis 1

1 2

Paula Stradins Clinical University Hospital, Riga, Latvia; Clinical Hospital Gailezers, Latvia

Objective: to evaluate safety and eficiency of endovascular treatment of intracranial aneurysms in children under 16 years of age. Materials and Methods: During 3 year period 4 patients with intracranial aneurysms under 16 year of age where treated. In all cases endovascular embolisation with platinum detachable coils was performed. 3 patients presented with SAH, one was unruptured aneurysm and patient was complaining about headache. There where two patients 15 years old, one patient 12 years old and one 2 years old. Younger child presented with ischemic lesion in basal ganglia and it was dissecting aneurysm. All other 3 patients had a single saccular aneurysms. Results: Complete obliteration of aneurysms were achieved in all cases, there were no intraoperative complications, no rerupture of aneurysm. All patients where discharged from hospital without neurological deficite. Follow up MR angio after 6 month untill now is done in 2 cases and showed no recanalisation of aneurysm. Conclusions: Endovascular treatment of pediatric intracranial aneurysms is reasonably safe and reliable method.

Background. This paper presents the potential and results of long­term complex neurorehabilitation in the case of child with multiple developmental anomalies on the basis of the author's own experience. Patient ­ boy with multiple inherited anom alies ­ cleft palate, cervi cal area ­ C1 dysplasie (anomalie of development), subluxation of C2 after C3, stenosis of spinal tract, missing of upper ribs (1st pair), foot deformities, stigmas in the face area. Following development disorders ­ psychomotor delay, stereotypic movements, signs of autism, subluxation of hips and knees, hearing loss (3rd level). Method. Methods of assessment ­ diagnostics by Vojta method, Munich Developmental diagnostic, video analysis. Neurorehabilitation was started from early age (4 month) till 7 years ­ regularly 4 month courses twice pro year. First complex included ­ therapy by Vojta method, physical therapy ­ handling etc., early age speech therapy. From 3.5 year were included musical therapy and Montessori therapy. Results. Patients achievements in the 7 years age ­ walking with support, full bowel and bladder control, adequate and purposeful work with methodical materials, interaction with other children and adults, adequate behaviour in the different situations in the everyday life. From 7 years child attend special programme of integrative education for children with special needs. Conclusions. It's very important to start early age neurorehabilitation for child with multiple development anomalies to promote quality of integration in the society. Keywords: multiple development anomalies, early age neurorehabilitation.

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

DOWN SYNDROME, EPILEPSY, THE BRAIN ABNORMALITIES; THREE CLINICAL CASES

Nataliya Smulska, Ludmila Chepiga

Paediatric Hospital, Kiev, Ukraine

Background. Epilepsy in childhood Down syndrome (DS) is considered to be not much higher then in the general population. Methods. We had in to the Department of Neurology 3 patients (two boys and one girl). They were born in time, had typical for DS dysmorphic features and karyotype with regular trisomy of chromosome 21 (47,XX,+21; 47,XY,+21). The development was delayed. 2 patients had infatile spasm (IS). The seizures began in the 4.5-th and 8-th months. The third patient had secondary generalized seizures with post seizures paresis in the right hand and leg along 10­15 minutes. Epilepsy began in the 1.5 years old. Results. EEG: first and second patients had hypsarrhythmia; the third had patterns of symmetrical (with accent in the left side) spike discharges and irregular slow activity. MRI: all patients had different the brain abnormalities. First ­ less volume of the cerebrum, second ­ periventricular atrophy and third ­ narrow and poorly developed superior temporal gyrus, small cerebellum and pons, agenesia corpus collosum. Then we began treatment (our patients have been antiepileptic drags ­ valproic acid and carbamasepin) the seizures were stopped. Conclusions. The children with DS can have the different type of seizures and many brain abnormalities. Abnormalities are present in the brain of persons with DS, and together with abnormal neuronal excitability they may lead to increased seizure susceptibility.

grades of IVH. While 71.4% of infants with IVH grade I­II were neurologically unimpaired, only 3 (25%) infants with IVH grade III­IV were normal, 5 (42%) patients de veloped psychomotor retardation, and cerebral palsy was diagnosed in 4 (33%) cases. Conclusions. Infants with IVH grade III­IV have poorer neurodevelopmental outcome compared to infants with IVH grade I­II. Long-term follow-up is necessary for children with IVH to detect their disabilities and to design appropriate therapeutic intervention.

A COMPARISON OF QUALITY OF LIFE OF MOTHERS WITH CHILDREN WITH/WITHOUT DISABILITIES: A SAMPLE BASED ON TURKISH POPULATION

Ugur Cavlak 1, Erdogan Kavlak 1, Ela Tarakci 2, Arzu Razak Ozdincler 3, Hakan Uysal 4, Suleyman Gursoy 1

1 2

Pamukkale University, Denizli, Turkey; Hacettepe University, Turkey; 3 Istanbul University, Turkey; 4 Eskisehir Osmangazi University, Turkey

Background: The aim of this study was to compare quality of life (QOL) of mothers of children with disabilities with mothers of healthy children. Method: Five hundred and eighteen mothers in the Western of Turkey region completed the Short Form Health Survey (SF-36). The mothers with children 8 to 312 months of age (190 mothers with physically disabled children, 182 mothers with intelligent disabilities children, and 146 mothers with healthy children participated in the study. The mean age of the mothers was 33.89±7,2 years (ranges: 18­68 years). Results: The motor developmental level of the children with disabilities described as follow; 43 apedal (11.5%), 87 quadripedal (23.3%), and 242 bipedal (65%). While significant differences among mothers in the following domains; "role limitations due to physical health", "energy and fatigue", "emotional well being", and "general health" were found, no significant differences were detected concerning "physical functioning", "role limitation due to emotional problems", "social functioning", and "pain". These results show that the mothers with physical disabled children had fewer scores of the SF-36. When the mothers compared based on the motor developmental level of the children, significant differences were found in domains of the SF-36 Survey such as; "energy-fatigue", "emotional well being", and "general health". Namely, the mothers with children with disabilities at apedal level had fewer scores of the SF-36 compared with mothers with children at bipedal or quadripedal level. Conclusion: Having a child with disabilities decreases QOL of the Turkish mothers. Also, the children at apedal level much more affect the QOL of the mothers than the children at bipedal or quadripedal level. The mothers with healthy children had better scores in domains of the SF-36 compared to the mothers with disabled children.

OUTCOMES OF INTRAVENTRICULAR HEMORRHAGE IN PRETERM INFANTS

Milda Dambrauskiene 1, Kristina Zaveckiene 1, Rugile Pilviniene 2

1

Kaunas University Hospital, Department of Pediatric Diseases, Kaunas, Lithuania; 2 Kaunas University Hospital, Department of Neonatology, Kaunas, Lithuania

Background. Intraventricular hemorrhage (IVH) is the most common brain injury in preterm infants that can have significant long-term effects on children's development therefore it is important to carefully monitor the neurologic outcome of these infants. The aim of our study was to assess the neurodevelopmental outcome in preterm infants with IVH. Method. The retrospective analysis of medical records of 134 preterm infants with cranial ultrasound abnormalities was performed. These infants were under observation at follow-up department until 12 months of corrected age. Results. Their mean gestational age was 29.31 weeks (range 23­36), mean birth weight 1398 g (range 603­3480). Ultrasound examination in the neonatal period revealed, that isolated IVH grade I­II accounted for 22.4% and in combination with periventricular leukomalacia (PVL) ­ 9% of cases, isolated IVH grade III-IV ­ 6.7% and in combination with PVL ­ 2.2%, PVL alone ­ 35.8%. During infancy, ventricular dilatation was found in 20% (5/24) of infants with IVH grade I, 44% (8/18) with IVH grade II and 25% (3/12) with IVH grade III­IV. Posthemorrhagic hydrocephalus was detected in 11% (2/18) of infants with IVH grade II and 75% (9/12) of infants with IVH grade III­IV (p<0.05), 5 of them (1 with IVH grade II and 4 with IVH grade III­IV) underwent a ventriculoperitoneal shunting. There were statistically significant differences in the outcomes among the groups with different

COGNITIVE OUTCOME OF CHILDREN WITH STROKE: A PROSPECTIVE STUDY

Margus Ennok 1, Anneli Kolk 2, Rael Laugesaar 2

1 2

Neurology Clinic of Tartu University Hospital, Tartu, Estonia; Children's Clinic of Tartu University Hospital, Tartu, Estonia

Background: Stroke in children is a rare occurrence that can significantly influence the development and cognitive maturation of affected children. Previous studies on different cognitive functions have given various results but have also shown

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more impaired performance of children with neonatal stroke (NS) compared with childhood stroke (CS) subjects. Not much is known about long-term changes in different cognitive functions. Our aim was to study cognitive profile and its change in children with stroke over the course of a 2-year period. Method: 20 children (13 with NS and 7 with CS) were tested on two occasions with NEPSY test battery (mean intertest interval was 2.29 years). NS children had a mean age of 6.86 on first and 8.64 on second testing, CS children had a mean age of 8.21 on first and 10.04 on second testing. Their cognitive performance was compared with groups of age and sexmatched control subjects from regular public kindergartens and schools. Results: On the first testing children with stroke had impairments in sensorimotor, visuospatial, language, memory and attention functions. NS children had a lower performance in visuospatial, sensorimotor and language skills while CS subjects had more marked memory problems. Most of these deficits were also revealed on second testing, showing impaired performance in the same subtests in sensorimotor, visuospatial, language, memory and attention domain. Difficulties in attentional inhibition became more marked over time. Significant improvement was noted in only some subtests. CS children improved on some memory tests while NS children improved in spatial construction task. Conclusions: Stroke in children leads to significant cognitive decline. Children with NS had more severe cognitive impairment than children with CS. Cognitive deficits seem to be persistent and children make only a limited recovery over a 2-year follow-up period. Results: LBP and ELM were found as common significant variables affecting the QOL of MDC regarding the following domains; GH, PF, and EWB. However, LBP and ELM were not found as significant variables for the SF domain. IEC and AM were found as significant variables for the SF domain. Conclusion: In our context, we found that LBP is the most common significant factor affecting QOL of the MDC in the sample. Since, MDC have to adapt to new roles, reorganize their lives and cope with care, parents with disabled children, especially mothers may benefit from an intensive family competence programme.

EVALUATION OF SPASTICITY AND TREATMENT SELECTION IN CHILDREN WITH CEREBRAL PALSY

Svajune Gradeckiene, Inga Siurnaite, Jolita Silanskaite, Milda Endziniene, Nerija Vaiciene

Kaunas University of Medicine, Kaunas, Lithuania

The management of spacticity and related motor disorders includes non-pharmacological, pharmacological and surgical methods. Long term management of spasticity in cerebral palsy (CP) should be based on objective criteria such as child's age, rating scales for spasticity, gross motor function and gait, goniometry, etc. Unfortunately, these criteria are not sufficiently applied not only in primary care but in specialized centers as well. Aim of the study was to evaluate the criteria used for the selection of spasticity treatment modalities and the control of their efficacy. Methods. The analysis of medical records of 79 patients from 1 to 17 years old with different spastic forms of CP was performed in four health care institutions of two cities in Lithuania. Results. Spasticity according to the Ashwort scale was measured just in 4 cases, gross motor function in 1. Oral muscle relaxants were administered to 39.2% of the patients, in most cases episodically, for less than 1 month. Effectiveness of muscle relaxants was not documented in 74.2% cases; in 25.8% of them the subjective opinion was given by parents or doctors. Splints were applied in 54.8% of cases. Botulinum toxin ­ A was used in 11 cases; in 8 of them injections were started at the age of 2­3 years but continued only in 4 patients. Objective measurements of spasticity were performed only in 1 case in this group. Orthopedic surgery was performed to 36.7%, the average age of the first operation was 6.4 years. Conclusions. The monitoring of spasticity and related motor functions as well as the choice and evaluation of treatment modalities are based on no objective criteria in most cases.

THE EFFECTS OF PHYSICAL AND SOCIAL HEALTH COMPONENTS ON QUALITY OF LIFE IN TURKISH MOTHERS WITH DISABLED CHILDREN

Ugur Cavlak 1, Erdogan Kavlak 1, Suleyman Gursoy 1, Ela Tarakci 2, Arzu Razak Ozdincler 3, Hakan Uysal 4

1 2 3

Pamukkale University, Denizli, Turkey; Hacettepe University, Turkey; Istanbul University, Turkey; 4 Eskisehir Osmangazi University, Turkey

Background: Parenting children who are physically or intellectually impaired is known to be linked to the impaired wellbeing of the parents themselves. Parents face a number of common tasks in managing their child's afflicted health. However, there is still little available data on quality of life (QOL) in parents of disabled children in Turkey. This cross-sectional study was conducted to describe the effects of some variables, which would interfere with the general health, on QOL of mothers with disabled children (MDC). Method: Three hundred and seventy two MDC living in the Western of Turkey region with a mean age of 34.73±7.5 years completed The Short Form Health Survey (SF-36). All gave their informed consent for participating to the study. We also recorded demographics of the MDC. The effects of 10 variables such as; motor developmental level of children, low back pain (LBP), gender of child, clinical type of disability of child, exercise habit of mother, educational level of mother (ELM), occupation of mother, involved extremities of child (IEC), age of mother (AM), and parity on four domains of the SF-36 including, general health (GH), physical functioning (PF), emotional well being (EWB), and social functioning (SF) were analyzed using backward multiple linear regression.

QUANTITATIVE ANALYSIS OF GAIT PATTERNS IN CEREBRAL PALSY

Zane Pavare 1, T. Kravchenko 1, O. Kudrjasovs 1, Zigrida Krumina 2, Anita Vetra 2

1

Gait laboratory of Rehabilitation Institute Riga Stradins University, Riga, Latvia; 2 National rehabilitation center `Vaivari', Jurmala, Latvia

Background. In this study we propose a quantitative analysis based on tree-dimensional gait analysis in order to evaluate visible impairments in the major joints of the CP children with spastic hemiplegia. Method. Gait analysis was preformed on 15 CP children. 9 of those were females and 6 were males within the age range of

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5 till 18. The clusters of 19 mm spherical retroreflective markers were placed bilaterally on the thighs and shanks lateral surfaces. In such way the motion range, joint moment and muscle energy was determined in three dimensions simultaneously in all of the patient's largest lower extremity joints by using a six Pro Reflex systems infrared video cameras of 240 Hz, AMTI force plate, 16 channel Delsys electromyograph. Joint kinematics and temporal parameters were calculated using Visual3D motion software. Results. The basic divisions of the gait cycle into stance and swing phase were normal. Changes were established in stance division: decreased single limb support in both legs, on the effected side for 5%, the unaffected side ­ 3%. During initial limb support there was increased plantarflexion moment at the ankle joint, decreased extension moment at the knee and as a compensation increased extension moment and power generation at the unaffected hip joint. There is a decreased ankle plantarflexion moment thereby resulting in an increased ankle inversion moment on the unaffected limb at the terminal single limb support. Conclusion. In CP children with spastic hemiplegia deviations of the stance phase and kinetic data were established in both legs. Decreased initial double limb support is associated with difficulties to load response, that require increased extension moment and power generation at the unaffected hip joint, decreased single limb support of the spastic side related with loss of stability, decreased terminal limb support ­ with difficulties to elevate the leg.

VAGUS NERVE STIMULATION (VNS) IN CHILDREN: TEN YEARS EXPERIENCE

Jorgen Alving 1, Hanne Nielsen 1, Kern Olofsson 2

1 2

Danish Epilepsy Center, Dianalind, Denmark; Department of pediatrics, Roskilde Hospital, Denmark

Aim of study. Tto evaluate the short- and long-term efficacy of VNS in children with intractable epilepsy. Effect parameters seizure reduction adverse reactions positive effects apart from seizure reduction. Patients. 25 children under age 18, all with difficult-to treat epilepsy, followed from 1996­2006. Seven were excluded from surgery after pre-operative work-up. All were mentally retarded, half of them severely. Epilepsy syndromes 17 localisation-related 8 generalised. Number of AEDs tried before VNS: 6­10. Four also had tried the ketogenic diet. Results. Seizure response. Seizure reduction by 50% or more: after 1 year: 6/25, after 2 years: 7/19, after 3 years: 7/15. One child died during follow-up. Failures in 2 children, VNS was switched off, and in another two, the stimulator as removed. Adverse reactions after 1 year (N = 25): voice alteration (7), increased seizure frequency (6), coughing (5), dyspnoea (3), pain at stimulation site (2), non-convulsive status (2), insomnia (2). Positive effects after 1 year (N = 25): Better mood (15), Improved attention (14), Successful aborting seizures (magnet) (7), shortened post-ictal phase (6). Conclusion. VNS is a safe, well-tolerated and effective treatment option for children with intractable epilepsy. The effect seems to be sustained over a prolonged period, and in accordance with previous findings in adults, to increase over time. The high frequency of positive effects on mood and attention is noteworthy.

EPILEPSY

TREATMENT OF INTRACTABLE EPILEPSY IN CHILDREN USING VAGUS NERVE STIMULATION: ALTERNATIVE MEDICINAL APPROACH

Anit Singh, Rajakrishnan V., Krishna Dalal, Manjiri Tripathi

All India Institute of Medical Sciences, New Delhi, India

SLEEP DISTURBANCES IN CHILDREN WITH EPILEPSY

Ruta Praninskiene 1, Danute Vysniauskiene 1, Jura Tuleviciene 1, Olga Kutuzova 1, Genute Markvaldiene 1, Aurelija Jucaite 2

1 2

Purpose: This study aims at managing patients suffering from intractable epilepsy by applying indirect vagus nerve stimulation (indirect VNS) through reflexology. Method: This is a 3 years randomized two-arm pilot study. The group in arm one is treated with pharmacological drugs, reflexology therapy and indirect VNS. The second arm comprising the control group continue on anti-epileptic drug therapy. Age group 3yrs to 45yrs, who had intractable epilepsy for at least 3yrs, met the inclusion criteria. Result: A total of 27 patients have been admitted to the study till March 2006. (Sample size of study group =13 and sample size of control group =14). The response of the patients to the therapy depended on good compliance. 80% of the patients showed a positive response with regards to reduction in seizure frequency, duration of ictal phase and improvement in behavioral patterns of patients, at the end of 4 months monitoring period. Conclusion: This is an ongoing study and the patients will be monitored for a period of 3 years. Unique response with Indirect VNS reveals the effectiveness of this technique in managing refractory epileptic patients with least side effect and cost, but with similar results as obtained in an expensive procedure of direct vagus nerve stimulation. Hence this complimentary medicine maintains uninterrupted personal and family lives.

University Children's Hospital, Vilnius, Lithuania; Karolinska Institutet, Institute of Woman and Child Health, Stockholm, Sweden

Introduction: Attention, learning problems in children with epilepsy can be attributed to their seizures as well as concomitant sleep problems. Thus, understanding of sleep disturbances (SD) in children with epilepsy may help to improve management of their neurocognitive deficits. The aim of the present study was to investigate the pattern of SD among children with epilepsy. Method: 197 children with epilepsy and their parents were asked to fill out the questionnaire for the SD among children (Bruni et al, 1996). The scale of sleep disturbances (SDSC) includes: difficulty in initiating and maintaining sleep (DIMS), sleep breathing disorders (SBD), arousal disorders (DA), sleep-wake transition disorders (SWDT), disorders of excessive somnolence (DOES), sleep hyperhydrosis (SHY). Results: 109 boys and 88 girls, 5.5 to 17.9 years of age participated in the study. The total SDSC scores, DA, SBD, SHY scores were higher in patients with active epilepsy and generalized seizures (p<0.05). Significantly higher SHY, SBD, SWDT, DA scores were obtained in the sub-group of children having just night seizures. Patients with Rolandic epilepsy have lower scores in DIMS (p=0.06) and DOES (p=0.07). Children with temporal epilepsy had lower scores in SBD (p=0.04). Gender effect was observed in DA and DOES scores (females having higher scores, p<0.02).

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Conclusions: The present study suggests that there is a close relation between seizures and sleep disorders and it is dependent on the seizure type and time, on the activity of the disease. The scale of sleep disturbances may be used to select children with epilepsy for the diagnostic overnight polysomnography test. Identification and treatment of these problems might improve quality of life of children with epilepsy. Methods. We studied the clinical, WEEG and SEEG findings on 121 patients with RE. These children were examined for RE and were treated at the Child Neurology Unit of Kaunas University Hospital from 01.10.2001 till 01.10.2003. Depending on the duration between the last seizure and the date of EEG recording, the patients were differentiated into 7 groups: Group 1 ­ < 1 week, Group 2 ­ > 1 week ­ < 1 month, Group 3 ­ from 1 to 3 months, Group 4 ­ from 3 to 6 months, Group 5 ­ from 6 months to 1 year, Group 6 ­ from 1 year to 2 years, and Group 7 ­ > 2 years. Results. When the period between the last seizure and the EEG recording is shorter, the probability of finding CTS in WEEG as well as in SEEG is higher (WEEG c2 =19.8, p<0.001; SEEG c2=20.4, p<0.001) than in a case of longer time period. CTS rate in SEEG was significantly different in groups of different time period between the last seizure and the EEG recording (Kruskal-Wallis ANOVA: c2=22.8, df=6, p=0.001), while CTS rate in WEEG in these groups was not statistically significantly different. Conclusion. The longer was the period between the last seizure and the day of EEG recording the lower was the probability of finding centrotemporal spikes in awake and sleep EEG (p<0.001) and the lower was the centrotemporal spike rate in sleep EEG (p=0.001) in patients with Rolandic epilepsy.

KNOWLEDGE AND ATTITUDE OF THE STUDENTS TOWARDS EPILEPSY

Giedre Jurkeviciene, Jurgita Vainauskiene, Milda Endziniene, Nerija Vaiciene, Giedre Gelziniene, Dovile Rackauskaite, Inga Simaityte

Kaunas University of Medicine, Kaunas, Lithuania

Purpose: to assess knowledge and attitude of medical and non-medical students towards the patients with epilepsy. Methods: there were 412 questionnaires filled by 280 (68.0%) students of Medicine Faculty, Kaunas University of Medicine, Kaunas, and 132 (32.0%) students of non-medical profile specialization from various universities of Lithuania. Questions were divided into 2 fields: 1) knowledge in epilepsy, 2) attitude towards the patients with epilepsy. Results: 249 (88.9%) medical and 95 (71.0%) non-medical students answered correctly that epilepsy is the disease of the brain. Non-medical students (15 (10.6%)) made a mistake considering epilepsy as a psychiatric disease more frequently than medical ones (12 (4.3%)), (p=0.01). They also more frequently did not know what ep i lepsy is (ac cord ingly: 14 (10.6%) and 6 (2.1%), p<0.001). All 5 proposed correct reasons of epilepsy were more frequently chosen by medical (84; 30.0%) than non-medical students (1; 0.8%), (p<0.001). Non-medical students (49; 37.1%) marked only one possible reason of epilepsy more frequently comparing with medical students (59; 51.1%), (p<0.01) and more frequently did not know the reasons, which cause epilepsy (accordingly: 47 (35.6%) and 30 (10.7%), p<0.001). All 4 proposed correct manifestations of epileptic seizure were more frequently marked by medical (99; 35.3%), comparing with non-medical students (3; 2.3%), (p<0.001). Non-medical students marked only one possible correct manifestation of epilepsy seizure more fre quently than med i cal stu dents (ac cord ingly: 67 (50.8%) and (61; 21.8%), p<0.001). The answers to the questions reflecting positive or negative attitude towards epilepsy were not statistically different between the groups of medical and non-medical students. Conclusions: 1. Medical students have better knowledge of epilepsy than non-medical ones. 2. Attitude of medical students after neurology and non-medical students did not differ towards the patients with epilepsy.

CENTRAL STIMULANT TREATMENT OF ADHD/ADD IN CHILDREN WITH EPILEPSY

Lene Sahlholdt, Lene Starup, Kirsten Juul, Tove Hoffmann, Ditte Jeppesen

Danish Epilepsy Hospital, Dianalund, Denmark

CHANGES IN EEG WITH DIFFERENT PERIODS BETWEEN THE LAST SEIZURE AND EEG RECORDING IN PATIENT WITH ROLANDIC EPILEPSY

Giedre Jurkeviciene, Nerija Vaiciene, Milda Endziniene, Viktoras Saferis

Kaunas University of Medicine, Kaunas, Lithuania

32 children, 9 girls and 23 boys, with epilepsy and ADHD/ADD were analysed in a retrospective study. Age 6 to 18 (mean 10 years). All received treatment with centralstimulants (31 methylphenidate, 1 methylphenidate plus modafinil) after neuropsychological evaluation, e.g. observation at the ward during admission and possibel testing. 29 were psychological tested, 3 were not testable. Full scale IQ were obtained in 25, IQ 40 to 117 (mean 72), IQ < 80 in 14. Centralstimulant treatment were introduced at the age of 5 to 15 (mean 8.5 years). 3 patients had ADHD hyperactive type, 11 ADD inattentive type, 13 ADHD combined hyperactive/inattentive type. Ep i lepsy clas si fi ca tion: 14 idiopatic gen er al ised (6 GTCS, 6 Absence, 1 JME, 1 MAE), 1 Epilepsy with Eyelid Myoclonus, 1 Epilepsy with Myoclonoc Absences, 1 Progressive Myoclonic Epilepsy, 2 Encephalopathy with CSWS, 2 Symptomatic multifocal, 5 Symptomatic focal, 2 BECTS, 3 Benign Occipital Epilepsy of Childhood, 1 unclassified. 29 were on antiepileptic drugs when treatment with centralstimulants were introduced. Monotherapy in 15, 2 drugs in 10, 3 drugs in 1, 4 drugs in 1. 22 were seizure free when centralstimulants were introduced, 7 still had seizures, information about 3 were not valid. There were no reports on relapse of seizures and no reports on incresed seizure frequency after introduction of centralstimulants. Sideeffects of centralstimulant treatment were reported in 16. The parents reported significant good effect of the centralstimulant treatment on daily life functioning in 30 children who thus continued the treatment, 1 stopped because of lack of effect, information is uncertain in 1. Conclusion: Treatment with centralstimulants in children with epilepsy and ADHD is safe and valuable in relation to the childrens function in daily life.

Purpose. To determine the probability of finding and the rate of centrotemporal spikes (CTS) in waking (WEEG) and sleep (SEEG) electroencephalograms depending on the time period between the last seizure and the EEG recording in patients with Rolandic epilepsy (RE).

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LONG-TERM TREATMENT OF COMPLEX PARTIAL SEIZURES STATUS EPILEPTICUS WITH INTRAVENOUS MIDAZOLAM: A CASE REPORT

Edita Kanyte, Jurate Grigoniene, Milda Endziniene, Nerija Vaiciene

Kaunas University Hospital, Kaunas, Lithuania

Patients with epilepsy may experience clusters of seizures sometimes progressing to convulsive or non-convulsive status epilepticus (SE). Refractory SE is defined as a condition that fails to respond to first- and second-line anticonvulsant medications. The management of repetitive seizures and refractory SE still lacks clear guidelines, and therapeutic approaches are mainly based on the individual experiences of treating physicians. We present 2-year-old previously healthy girl who at the age of 9 months started with frequent partial seizures with secondary generalization characterized by deviation of the eyes to the right, tonic strain, short clonic jerks of the limbs and followed by postictal prolonged sleep. VideoEEG showed intensive epileptiform discharges in the left frontal-temporal region. CT and MRI were normal. Seizure control was achieved for 3 months with valproate and nitrazepam. Later short seizures manifesting with a cry, tonic strain of arms, vegetative symptoms, frightful look, impaired consciousness relapsed, and the EEG revealed continuous spike-slow wave discharges in the left frontal-central-temporal region and less intensive discharges in the parallel right side. The additional therapy was disappointing: sultiam, corticosteroids, high doses of topiramate (15 mg/kg/d) failed, carbamazepine induced a worsening and lamotrigine caused the hypersensitivity syndrome. Eventually seizures in clusters developed that progressed to SE of complex partial seizures. Whereas the initial treatment of SE with intravenous diazepam, phenobarbital and phenitoin failed, the permanent infusion of midazolam was initiated. The dosage had been increased from 1 to 8.3 microg/kg/min until seizures stoped, then gradually was reduced and discontinued after 18 days. The treatment was tolerated very well, neither cardiorespiratory depression nor sedation occured. Only single seizures have been occuring for the past 6 months. Permanent intravenous midazolam is worth a try when basic anticonvulsant therapy is not able to protect from SE and classic anticonvulsant medications fail.

while children without epilepsy projected emotions also towards environment (CWE depicted anger as placed inside, those without epilepsy represented situational drawing; `Unhappy Snail' lived in the closed off house according to the most drawings of CWE, to compare with just 30% of the control group). CWE were timorous to compare with the control group (drawings are more faint, from one line, scarce experiments with intense colors, simple structures), more lonely (phenomenon of one person, one flower, predominate in the drawings), diffident (the person drawn is with dropped eyes, hands, without feet), more static (in drawings of action, environmental object are immobile), feeling overprotection from his family (in drawings other family members are bigger than him/her), less interested in environment (background in the drawings are not filled up, structures are very poor, roughcast, not painted). Conclusions: The drawings of CWE reflected perceived stigma which was especially obvious in the older age group.

GENETIC AND NEURODEGERATIVE DISORDERS

ENVIRONMENTAL FACTORS IN THE DEVELOPMENT OF AN ACUTE RHABDOMYOLYSIS IN THE PATIENT WITH THE SHORT-CHAIN ACYL-COA DEHYDROGENASE GENE POLYMORPHISM 625 G>A

Birute Skerliene 1, Marija Jakutovic 1, Donatas Petroska 1, Willy Lehnert 2

1 2

Vilnius University, Vilnius, Lithuania; Freiburg University, Germany

Short-chain acyl-CoA dehydrogenase (SCAD) is the first enzyme of the mitochondrial short-chain beta-oxidation spiral catalyzing the dehydrogenation of C4 and C6 fatty acids. Inborn SCAD deficiency is defined and belongs to the group of the inherited fatty acid oxidation defects. In addition to pathogenic or inactivating mutations, which are responsible for severe diseases, two polymorphisms have been identified which are common in the general population. Individuals carrying the 625 G>A polymorphism are at risk of developing symptoms. Objective: other genetic, cellular and environmental factors are considered to be involved in reducing the level of catalytic activity of the variant enzymes below a critical level, leading to the onset of clinical symptoms. Material and results. A patient with 625 G>A polymorphism is presented who experienced episodes of acute rhabdomyolysis since the age of 3.4 years. Investigations for the other genetic and inflammatory muscle diseases were negative. The last episode of an acute rhabdomyolysis resulted in the lethal outcome because of ventricular asystoly due to hyperkalemia. Discussion. A deficiency may either be consistently expressed or related to intermittent cellular stress. Both variant proteins at higher temperatures may result in insufficient amounts and activity and thus the development of SCAD deficiency. Reduced pH, high fatty acid oxidation activity may result in accumulation of butyric acid which is cytotoxic. Ethylmalonic acid is supposed to be responsible for the inhibition of the electron transport chain and creatine kinase activity in human skeletal muscle. Conclusion: the severe course of the disease in the patient was perhaps determined by an unusual physical loading, fasting, high weather temperature and other unfavourable conditions.

PERCEIVED STIGMA IN DRAWINGS BY CHILDREN WITH EPILEPSY

Gintare Zukauskaite, Milda Endziniene

Kaunas University of Medicine, Kaunas, Lithuania

Children with epilepsy (CWE) may experience difficulties in social integration which is not easy to identify by verbal communication. Aims: To identify the peculiarities of self-perception of CWE. Methods: We analyzed the drawings by 210 CWE and of 270 children with other neurological disorders (control group), hospitalized at Child Neurology unit, Kaunas University Hospital, 3­7 years and 8­17 years of age. Seven topics were suggested to all participants: Myself (I am real, Myself as a symbol), Disease, Epilepsy, Time, Place, Emotion, Activity. Results: in 70% cases of 3­7 years old CWE warm colors dominated, in 95% of children of 8­17 years old cold colors did. In most cases CWE identified `Myself' with the disease, accepted own disease, but could not accept himself/herself (clear details of the child, clear face of the disease, projection of the disease in oneself). CWE projected emotions to oneself

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

DOPA-RESPONSIVE DYSTONIA ­ A CASE REPORT

Inga Talvik, Kadi Veri, Tiina Talvik

Children's Clinic of Tartu University Hospital, Tartu, Estonia

Coloboma of the iris was found in the mother and obscure signs of myotonia in the otherwise healthy father. The treatment with phenytoinum was started and is still continued with the doubtful positive effect till her recent 12 years of age. Discussion and conclusions: SJS can be not as rare as misdiagnosed; clinical and genealogical peculiarities were disclosed; molecular genetic study should be performed in order to contribute to the feather research of this pathology.

Dopa-responsive dystonia (DRD), first described in 1976 by Segawa, is a rare hereditary disorder ­ progressive dystonia with diurnal fluctuations. The most common form of dopamine-deficit syndrome is an autosomal-dominant condition caused by mutation of the gene for guanosine triphosphate (GTP) cyclohydrolase I. In classical cases the disease manifests in early childhood with dystonia of the lower limbs and there is dramatic and sustained response to treatment with levodopa. Several authors believe that DRD is an underdiagnosed condition and frequently misdiagnosed as cerebral palsy ­ "striatal toe" may be misinterpreted as a positive Babinski sign. We describe a 11-year-old girl with a 2 year history of progressive walking difficulties. Diurnal variation of her symptoms were observed ­ the gait disturbance became worse during the day and after exercise. Her perinatal and family history was unremarkable. Clinical examination revealed brisk tendon reflexes, equinovarus posturing of the left foot and a contracture of the left ankle. Brain computed tomography scan and lumbosacral magnetic resonance imaging were normal. Twenty-four hours after starting treatment with low dose combined levodopa and carbidopa a dramatic improvement was noted in her dystonic gait. On follow-up one month later there was barely detectable gait disturbance. The patient continues replacement of dopamine using oral levodopa 4 mg/kg/day and physiotherapy. Dopa-responsive dystonia should be considered and empiric treatment with levodopa should be started in any child who presents with progressive dystonia of unkown etiology.

ANGELMAN SYNDROME: A CASE REPORT

Svajune Gradeckiene 1, Danielius Serapinas 1, Milda Endziniene 1, Inga Bucinskiene 2, Jolanta Jeroch 2, Daiva Urboniene 2, Nerija Vaiciene 1

1

Kaunas University Hospital, Neurology Department, Kaunas, Lithuania; 2 Kaunas University Hospital, Genetic Counceling Unit, Kaunas, Lithuania

CLINICALLY CONFIRMED SCHWARTZ-JAMPEL SYNDROME TYPE I

Birute Skerliene, Danute Vysniauskiene

Vilnius University, Vilnius, Lithuania

We report a case of genetically confirmed Angelman syndrome, described in 1965 by Angelman. A 7-year-old girl was referred to the Pediatric Neurology Unit in March 2006 for consultation about possible treatment with botulinum toxin A for her cerebral palsy. At the age of 6 years she had generalized tonic clonic seizures provoked by high fever and considered as febrile ones. Her clinical examination showed severe mental retardation with marked delay in attainment of motor milestones, paroxysms of inappropriate laughter, blond hair, ocular anomalies, including decreased pigmentation of the choroid and the iris, the latter resulting in pale blue eyes, jerky arm movements resembling a puppet gait and hypermetropia ­ symptoms which clin i cally were char ac ter istic to Angelman syndrome. Sleep EEG showed high amplitude 2.5­3 Hz spike waves generalized activity, episodic asymmetric delta slow spike waves activity in the right frontal region. Antiepileptic drug treatment was started. The patient was referred for genetic consultation with suspicion of Angelman syndrome. Cytogenetic-molecular analysis revealed deletion of 15q 11 chromosome which is typical for Angelman's syndrome. Conclusion: This case demonstrates the successful application of newly implemented cytogenetic molecular investigation at Kaunas University Hospital which helps to detect previously unknown causes of neurological disorders.

Schwartz-Jampel syndrome (SJS) is a very rare inherited disease with a distinctive association of myotonia and chondrodysplasia. The pathophysiological mechanisms of SJS are only recently started to be recognized. SJS results from mutations in the HSPG2 gene encoding a major component of basement membranes perlecan, in contrast to the other myotonic disorders which arise from mutations in genes encoding voltage-gated ion channels. Until recently, very few patients had been studied genetically. Objective: a patient with this rare and insufficiently investigated disease is presented. A 5.5 years old girl with the diagnoses of epileptic syndrome and cerebral palsy was admitted to the hospital in order to specify the reasons of the joint stiffness where after the discovery of the irregularities of the capital femoral epiphyses and vertebra. Myotonia, blepharospasm, diminished muscle mass, thigh hypertrophy, hyporeflexia, skeletal dysplasia (high-arched palate, micrognatia, short neck, pectus carinatum, coxa valga, pes planovalgus, kyphoscoliosis, reduced stature), were disclosed. Myotonia presented as painless tonic spasms or temporary rigidity of the muscles after an active or passive attempt was made to move them since the age of 1.5 month. Joint hypermobility was more characteristic during relaxation of the muscles except some fixed contractures. ENMG showed continuous electrical activity and normal nerve conduction. Illnesses of the mother during pregnancy (influenza and treatment due to cardiologic complications) were disclosed.

INFANTILE GM1 GANGLIOSIDOSIS: FATAL DISEASE DUE TO INHERITED PROGRESSIVE NEURONAL DAMAGE

Algirdas Utkus 1, Chandra Ward 2, David Booth 2, Anthony Fensom 2, Birute Tumiene 1, Jurate Grigoniene 3, Vaidutis Kucinskas 1

1 2

Vilnius University, Vilnius, Lithuania; Biochemical Genetics Laboratory, Genetics Center, Guy's Hospital, UK.; 3 Kaunas University Hospital, Department of Neurology, Kaunas, Lithuania

GM1 gangliosidosis is a lysosomal storage disease caused by mutations in a GLB1 gene and a deficiency of enzyme beta-galactosidase. Accumulation of undegraded gangliosides occurs in the neurons, forming typical meganeurites, and in the visceral organs. Our presented patient was a 1.5 years old female with typical symptoms of infantile GM1 gangliosidosis. She was born to non-consanguineous parents of Ukrainian and Russian descent after an uneventful pregnancy per sectionem cesaream. Edema in lower ex-

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tremities and in the periocular region was observed after delivery. Appetite was good and weight gain was normal. Increased irritability, sensitiveness to sounds and fine horizontal nystagm were noted from birth. From the age of two months infantile spasms were observed: stiffness, redness in face and apneic episodes started at the time of crying or exercises and lasted for several seconds. There was developmental regress from very early in infancy: the child had a head control at the age of 3.5 months but later lost it, smiling appeared only at 11 months, gaze fixation ­ at the age of 12 months of age. CT scan of the head region was performed in Kaunas University of Medicine Clinics: hypoplasia of vermis cerebelli and secondary vetriculomegaly was observed in it. Typical symptoms of cherry-red spots were found in ocular fundi. Phenotype at the age of 1.5 years was also suggestive: profoundly impaired psychomotor development, almost no active movements, macrocephaly, coarse facial features, gingival hyperplasia, no teeth, macroglossia, hypertrichosis, hepatomegaly, abducted great toes in both feet. Diagnosis of GM1 gangliosidosis was confirmed in the Genetics Center of Guy's Hospital in London: characteristic abnormal bands were observed after the evaluation of urinary oligosaccharides by thin layer chromatography and grossly diminished activity of beta-galactosidase was determined in blood leucocytes.

MISCELLANEOUS

NEUROLOGICAL MANIFESTATIONS OF PEDIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS WITH ANTIPHOSPHOLIPID SYNDROM: A CASE REPORT

Asta Judickiene, Ruta Praninskiene, Marija Jakutovic

Vilnius University Children's Hospital, Vilnius, Lithuania

HALLERWORDEN­SPATZ DISEASE: CASE REPORT

Tatjana Volkova, Nerija Vaiciene

Kaunas University Hospital, Kaunas, Lithuania

Hallerworden ­ Spatz disease (HSD) is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia, first described in 1922 as a form of familial degeneration characterized by iron deposition in the brain. The usual onset is in late childhood or adolescence. Pathologic evaluation reveals characteristic rust-brown discoloration of the globus pallidus and substatia nigra pars reticulata secondary to iron deposition. Clinical manifestation is variable, usually starting with movement disorders such as rigidity of extremities, slowness of movements, dystonia, choreoathetosis and tremor. Dysphagia and dementia as well as visual impairment due to optic atrophy or retinal degeneration can be presenting symptom in many cases. No biochemical marker has been found in HSD. MRI pattern is fairly diagnostic for HSD and has been termed the "tiger's eye" sign: bilateral symmetric hyperintence signal in anterior medial globus pallidus with surrounding hypointensity in the globus pallidus and substantia nigra pars reticulata on T2-weighted images. We present a boy who came to the medical attention at the age of 2 months because of motor delay and hyperactive behaviour since 3 years old. At the age of 3 years incipi ent atro phy of opti cal nerve and pigmental retinitis were found. Initial extrapyramidal symptoms started at the age of 7 years and rapidly progressed with development of painful dystonias and dysphagia followed by dementia. Tests for Wilson's disease, acanthocytosis, lipoid amino acids were negative. Initially CT and MRI did not reveal any lesions. The "tiger's eye" sign on MRI was defined at the age of 9 years. The attempt to reduce dystonias with Sinemet, Baclofen and benzodiazepines were of very limited effectiveness. The patient died at the age of 10.5 years. Conclusion: HSD should be suspected in case of rapidly progressing dystonia starting in childhood. The typical MRI pattern in a presence of clinical symptoms is pathognomonic for HSD.

Systemic lupus erythematosus (SLE) is a chronic, inflamatory autoimune disorder. It may affect the skin, joints, kidneys, brain and other organs. The central nervous system (CNS) involvement is a common but rarely discussed feature of pediatric systemic lupus erythematosus, particulary with secondary antiphospholipid syndrome (APS). The antiphospholipid syndrome is a thrombophilic condition manifested by vascular thrombosis with the presence of antiphospholipid antibodies. The spectrum and severity of CNS manifestations of SLE varies widely, from stroke or seizures to more subtle, abnormalities in neurocognitive functions, such as attention, memory and learning. Thus, the disease must be diagnosed as early as possible. We report a case of an 11-year-old boy in whom disease manifested with hemichorea, seizures (1 episode) and behavioral aberrations. Electroencephalography showed unspecific discharges in the right hemisphere. Carotid duplex Doppler ultrasonography, transcranial Doppler ultrasonography and brain computed tomography ­ without changes. Brain magnetic resonance imaging showed the features of vasculopathy on both sides of parietal region. Anticardiolipin antibodies and anti-beta-2 glicoprotein-1 antibodies were present. Activated partial thromboplastin time (APTT) was prolonged. Positive antinuclear antibodies and low levels of complements C3, C4 were found. In our case the correct diagnosis was made 3 weeks after the disease onset. The patient was treated with prednisolone, cyclophosphamide, acetylsalicylic acid and sodium valproate. His chorea gradually improved and completely disappeared after 2 months, no more seizures occurred, behavioral abnormalities partially resolved. APTT decreased to nearly normal time. Conclusions. SLE is to be considered in the differential diagnosis of many neurological conditions. Early diagnosis of the disease may improve clinical management and prognosis. A timely and effective management of SLE and prevention of complications may be achieved by the early detection of APS.

RELATIONSHIP BETWEEN LYME BORRELIOSIS AND PERIPHERAL FACIAL PALSY IN CHILDHOOD

Daiva Savickiene, Ruta Praninskiene, Irena Narkeviciute, Janina Sinkeviciene, Danute Vysniauskiene, Jura Tuleviciene

University Children's Hospital, Vilnius, Lithuania

Introduction. Lyme borreliosis is a multisystemic disorder characterized by several clinical stages. Peripheral facial palsy (PFP) may be the only sign of Lyme borreliosis in children. This facial palsy requires the diagnostic differentiation from Guillain-Barre syndrome, Miller-Fisher syndrome, and especially idiopathic facial palsy (Bell's palsy). The aim of our research was to investigate the main etiopathogenic factors of PFP in children, especially in relation to Lyme borreliosis. Methods. The prospective study was performed in the Vilnius University Children's Hospital, Department of Neurology. We have tested 58 cases of children with PFP in the period from

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September 2004 year to June 2006 serological for neuroboreliosis enzyme ­ linked immunosorbent assay (ELISA, Behring, Germany), and Western blotting (WB). Results: 32 boys and 26 girls, 1 to 18 years of age participated in the study. Borreliosis was confirmed in 10 (17%) of patients (3 boys and 7 girls, from 4 to 17 years of age). PFP was observed in 4 cases on the right side and in 6 ­ on the left side. Only 2 of the children and their parients remembered having a tick bite. All the patients didn't have history of erythema migrans. All the children became ill in May ­ October period. Conclusions: The present study suggests that B.burgdorferi as ethiopathogenic factor in children with PFP was confirmed in 17% of cases. Only 3.4% of patients stated having a tick bite and nobody has observed erythema migrans. in 10­13 years. Males are 2­4 times more likely than females to develop a germ cell tumor, but there is a predilection for suprasellar tumors to occur in females. An 11 years old girl came to our clinic with disturbed vision, diabetes insipidus and lethargy. CT scan observed obstructive hydrocephalus and tumor in hypothalamus region. Stereotactic biopsy confirmed germinoma. Alpha- fetoprotein and beta-human chorionic gonadotropin were negative. Improvement was achived by placement of ventriculoperitoneal shunt. Radiotherapy 56 Gy and two courses of BEP (bleomycin, ethoposide, and cisplatin) were administered to this patient. Afterwards the mother refused to treat her child because of complications of chemotherapy. Six months later this girl came with the tumor within the abdomen 6×5 cm close to the outgoing ventriculoperitoneal shunt. The operation was performed and 5 chemotherapy (BEP) courses were given. Now the girl is in complete remission with normal vision and second-string therapy for panhypopituitarism.

TICK-BORNE ENCEPHALITIS VIRUS NEUROINFECTIONS IN CHILDREN

Jurgita Grikiniene, Renata Mazeikaite

Vilnius University Children's Hospital, Vilnius, Lithuania

ROSAY-DORFMAN DISEASE PRESENTING AS A TUMOUR OF HYPOTHALAMUS REGION

Rosita Kiudeliene, Giedre Rutkauskiene, Liutauras Labanauskas

Kaunas University Hospital, Kaunas, Lithuania

Background: Lithuania is endemic region of two most common tick-born diseases ­ tick-borne encephalitis (TBE) and Lyme disease. TBE is a serios neuroinfection in adults with high ratio of neurological sequelae. TBE is believed to be a benign disease in childhood. The aim of the study was to eveluate peculiarities of TBE virus neuroinfections in children. Methods: we retrospectively analysed case histories of all children diagnosed wih TBE in Vilnius University Children`s Hospital in 2003­2005. Results: we analysed 16 cases (3 girs and 13 boys), aged 13 ± 3 years. Tick bite was known in 14 cases (88%). After an incubation period of 11 ± 9 days biphasic course of the disease was in 11 cases (69%). An interval between first and second stages was 10 ± 4 days. The second stage manifested as acute meningitis. All cases were serologically confirmed: Ig M antibodies of TBE virus were positive. Ig G antibodies were pos i tive in 10 chil dren (63%). Blood leucocytosis (14.5 ± 3.02 × 109/l) and granulocytosis (80.6 ± 5.7%) were found in 15 case (94%). Elevated ESR (26.2 ± 17.5 mm/h) and CRB (14.3 ± 16.6 mg/l) were found in 12 cases (75%). Cerebrospinal fluid (CSF) cytosis was from 54 to 656/µl (164 ± 154/µl) with a predominance of lymphocytes in 14 cases (88%). CSF proteine was elevated (0.71 ± 0.38 g/l). 14 children (88%) were treated with short course (average ­ 4 days) of glucocorticoids. The hospitalization lasted from 5 to 12 days. All patients had a benign outcome without lasting neurological deficits or necessity for rehabilitation. Conclusion: all studied TBE virus neuroinfections cases presented with isolated meningitis serosa of moderate severity. None of the children studied had lasting neuro logi cal sequelae.

Rosai-Dorfman disease ­ RDD (sinus histiocytosis with massive lymphadenopathy) is an idiopathic histiocytic proliferative disease characterized by painless massive cervical lymphadenopathy, fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal gammopathy. Involvement of variety of extranodal sites is common ­ skin, bone, upper respiratory tract, and salivary glands. Involvement of the central nervous system (CNS) by Rosai ­ Dorfman disease is unusual and isolated CNS disease in the absence of nodal disease is distinctly rare. We present a case of extra nodal RDD manifesting primarily as diabetes insipidus and tumor in hypothalamus region. The patient was 12 year-old girl who presented with clinical signs of diabetes insipidus. Serum thyroid hormone and hydrocortisone levels were low. MRI scan showed a 0.9×0.9×1.0 cm tumor in the hypothalamus region. The patient underwent subtotal resection of the tumor. Biopsy from a mass in the hypothalamus demonstrated inflammatory proliferation with S100 negative, CD1a neg a tive, CD 68 pos i tive histiocytes show ing signs of emperipolesis. Local radiotherapy 12 Gy was given. After 9 month girl is in remission without proliferation of the disease.

RECURRENT PAIN AND USE OF ANALGESIC DRUGS AMONG SCHOOLCHILDREN IN LITHUANIA

Donata Jurgaityte, Laura Brauklyte, Liauda Jasionyte, Nerija Vaiciene

Kaunas University of Medicine, Kaunas, Lithuania

A CASE OF CNS GERMINOMA IN AN 11 YEARS OLD GIRL

Rosita Kiudeliene, Giedre Rutkauskiene, Liutauras Labanauskas

Kaunas University Hospital, Department of Pediatrics, Lithuania

Germ cell tumors, the most common type of tumor in the pineal region comprise a heterogeneous group of primary brain tumors arising from pluripotential germinal cell. The most common is the germinoma, accounting for 40­60% of all germ cell tumors. This tumor account for 0.4­3.4% of intracranial neoplasm. More than 70% of germ cell tumors present

Background. Recurrent pain is one the most often complaint of the children. The aim of this work was to investigate prevalence of the recurrent pain among schoolchildren, the most frequent localizations and the intensity of the pain, provocative factors, interference with daily activities and the use of analgesic drugs. Method. 456 schoolchildren of 10­18 years old were asked to fill in the original anonymous questionnaire. All children were chosen incidentally in 3 different regions of Lithuania. Results: 83.5% of children indicated that they suffered recurrent and troublesome pain; headache was the most frequent

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among them. Pain interfered with the daily activity in 86.8% of children. The average estimation of the pain was severe for girls and moderate for boys. Mental exhaustion and emotional stress in school were mostly pointed out as the possible provocative factors of the pain. Two-thirds (66.4%) of pupils characterized themselves as sensitive and nervous, 64.3% as constantly tired and having not enough sleep; 46.4% of children indicated that the recurrent pain lasted more than 1 year, and 17.8% ­ more than 2 years. The most of children (33.3%) indicated frequent recurrence of the pain up to several times per week. Only 42.2% of children were consulted by doctor for their pain, 60.1% were using analgesic drugs, only half of them prescribed by doctor. The most often used analgesics indicated by 158 children: paracetamol 21.2%, metamizol natrium 14.6%, 32.9% ­ the combined tablets of acetylsalicylic acid, paracetamol and caffeine. Conclusions. More than 4/5 of schoolchildren indicated having recurrent pain, mainly headache, which interfered with their daily activity. In most cases they were not consulted by a doctor, most of them were using not licensed for children analgesics. More awareness about childhood pain problem is needed in primary care as well as in the society and the family.

DYNAMICS OF CHILDREN NEUROPHYSIOLOGICAL PATTERNS (EEG, BERA) DURING BRAIN COMA AFTER SEVERE TRAUMATIC BRAIN INJURY

Ruta Liesiene 1, Ingrida Uloziene 2, Vytautas Ragaisis 3

1 2

Kaunas University of Medicine, Kaunas, Lithuania; Laboratory of Neuroscience, Institute for Biomedical research, Kaunas University of Medicine, Kaunas, Lithuania; 3 Unit of Children Neurosurgery, Department of Neurosurgery, Kaunas University of Medicine, Kaunas, Lithuania

Objectives: The aim of study was to evaluate and compare the predictive powers of clinical examination combined with EEG and studies of BERA results in determining the prognosis in traumatic coma. Method: Evaluation of coma using Glasgow coma scale (GCS); EEG mapping; BERA. Results: total of 43 children with severe acute head trauma were included in the study. Supratentorial injury was established as most frequent type of injury, affecting in 84% of cases. Intraventricular, parenchimal, subarachnoidal hemorrhagies were noticed in 72%, brain edema in 91% of cases. Majority of children (35) survived, 8 were dead. The most frequent mortality (38.5%) was amongst the group of children with GCS 3 in admission. In 19 patients (49%) during EEG monitoring were observed constant or intermittent slow wave activity, low amplitude of arrhythmic activity and local slowing of activity corresponding to brain damage seen on CT. For 17 patients BERA were elicited. In 4 cases it was abnormal due to acoustic nerve permeability disturbance at the level of pontine region. In 13 cases no disturbances of permeability was monitored from the level of cochlea to intermediate brain, but consciousness recovered after 19 and 24 days.Clinical recovery of consciousness 1 month after trauma was monitored in 29 cases, 6 patients remained in coma (14%).Continuation of clinical data and neurophysiological investigations will supporting analysis of prognostic factors and possible outcomes. Conclusion: Abnormalities on CT and marked EEG not always had abnormal outcome. Abnormal BERA correlated with EEG abnormalities and brain lesions seen on CT scan was followed by duration of coma for more than 1 month. Based on the present data and a literature review combined clinical examination with results of EEG and BERA can be used to establish early prognosis in a child traumatic brain injury.

TWO CASES OF CAT SCRATCH DISEASE ENCEPHALOPATHY PRESENTING WITH STATUS EPILEPTICUS

Jurgis Strautmanis, Inese Kazaine

Children's Clinical University Hospital, Riga, Latvia

Background. Cat scratch disease (CSD) is a world wide, non-epidemic infection caused by the Gram negative bacillus Bartonella henselae. Typical presentation consists of rash, regional lymphadenopathy and general symptoms (fever, headache, malaise). CNS findings occur in 5­10% of cases and status epilepticus is the most frequent emergency of CSD encephalopathy. Case reports. 9 year old girl was admitted to small regional hospital at 27.10.2006 with complaints on fever and regional lymphadenopathy of right hand. The patient was scratched by kitten a month before admission. 5 days later developed status of generalized tonic clonic seizures and the patient was transferred to Childrens Clinical University hospital in Riga. On admission fever, generalized lymphadenopathy (most prominent in right axillary region), conjunctivitis and corneal erosion of eyes, unconsciousness, positive Babinski sign and meningeal signs were present. Seizures were controlled with Thiopental. 2 days girl was unconscious, but mental status gradually improved. Subsequently developed ataxia, tremor and cogwheel rigidity that disappeared within 2 months on Amantadine. CSF, MRI was normal and various serological, bacteriological and virology tests were negative. Indirect fluorescent antibody test to B.hehselae was also negative. Lymph node biopsy and skin testing wasn't performed. Two months later 3 years old girl from the same family was hospitalized with similar clinical presentation. She had had contact with the same kit ten. In di rect flu o res cent an ti body test to B.hehselae was also negative. Con clu sions. There are no pre vi ous re ports of CSD encephalopathy in Latvia. Although we couldn't prove diagnosis by serological tests we suppose that these cases could be referred to CSD because of combination of history (contact with kitten), clinical presentation (regional lymphadenopathy with status epilepticus), lack of changes on CSF examination and MRI and benign outcome. Negative serology test could be explained by existence of different B.henselae serotypes.

UNUSUAL CLINICAL PRESENTATION OF GANGLIONEURINOMA LOCATED IN THE COSTO-VERTEBRAL JUNCTION

Alla Nechay 1, Tatyana Stetsenko 2, Ludmila Chepiga 1

1 2

Paediatric Hospital No 1, Kiew, Ukraine; Academy of postgraduate training, Ukraine

A case report of the 3 year old girl with ganglioneurinoma in the costo­vertebral junction is presented Since aged 2y4m the girl developed sleepiness, sluggish movements and constipaption. At the time of presentation her sleep was deep, she did not awaken on her own. When awake she could unexpectedly lose her muscle tone and fall down, sometimes she would laugh without context and lose her tone at the time of such laughter. She appeared weak with ptosis, muscular hypotonia and losing the possibility for active movements. Myoclonias were seen during sleep and awakening. Nevertheless the girl did not lose her interest in toys nor for studying new things. Symtoms progressed rap-

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

idly; periodi cally tachycardia and tachypnoea appeared. Blood count, routine biochemistry and urine were normal. EEG did not show epileptiform discharges. ECG was normal. Brain MRI did not show any ab nor mal i ties. EMG showed a myasthenic reaction of Lambert­Eaton type. MRI of mediastinum revealed a tumor of sympathic ganglia 65­19.3 mm in the left costo-vertebral junction at the level of D5 ­ D9, that took the contrast non ­ homogeneously, ganglioneurinoma was presumed. The case report demonstrates that hypersomnia with paroxysmal events and my asthenic syndrome may be caused by tumor of mediastinum.

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INDEX OF AUTHORS

9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

A

Aksiks Igors . . . . . . . . . . . . . . . . . . . . . 30 Alving Jorgen . . . . . . . . . . . . . . . . . . . . 33

H

Hoffmann Tove . . . . . . . . . . . . . . . . . . . 34

I B

Baiardi Paola . . . . . Barbon Galluppi Renza Basel-Vanagaite Lina . Beni-Adani Liana . . . Ben-Sirah Liat . . . . . Biani Naresh . . . . . . Boltshauser Eugen . . Booth David . . . . . . Brauklyte Laura . . . . Brumback Roger . . . Bucinskiene Inga . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26 26 24 19 19 19 24 36 38 26 36 Istigeceva Liuda . . . . . . . . . . . . . . . . . 18, 30

J

Jakutovic Marija . . Jasionyte Liauda. . Jeppesen Ditte. . . Jeroch Jolanta . . . Jovic Nebojsa . . . Jucaite Aurelija . . Judickiene Asta . . Jurgaityte Donata . Jurkeviciene Giedre Juul Kirsten . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28, 35, 37 . . . . 38 . . . . 34 . . . . 36 . . . . 21 22, 29, 33 . . . . 37 . . 20, 38 . . . . 34 . . . . 34

C

Caplan Rochelle . . . . Cavlak Ugur . . . . . . Ceci Adriana . . . . . . Chepiga Ludmila . . . Constantini Shlomi . . Covanis Anthanasios . Cvitanovic Sojat Ljerka . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22-23 28, 31-32 . . . . 26 . . 31, 39 . . . . 19 . . . . 23 . . . . 27

K

Kanyte Edita . . . . Kaulina Aija . . . . Kavlak Erdogan . . Kazaine Inese . . . Kidikas Helmuts . . Kiudeliene Rosita . Kolk Anneli. . . . . Kravchenko T. . . . Kretaine Agnese . . Krumina Zigrida . . Kucinskas Vaidutis Kudrjasovs O. . . . Kupcs Karlis . . . . Kuske Sandra . . . Kutraite Asta . . . . Kutuzova Olga . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35 . . 30 31-32 . . 39 . . 30 20, 38 22, 31 . . 32 . . 30 . . 32 . . 36 . . 32 . . 30 . . 30 . . 28 . . 33

D

Dalal Krishna. . . . . . . . . . . . . . . . . . . . . 33 Dambrauskiene Milda . . . . . . . . . . . . . . . . 31 Dumalakiene Irena . . . . . . . . . . . . . . . . . 29

E

Endziniene Milda . . . . . . . . . . . 27-29, 32, 34-36 Ennok Margus . . . . . . . . . . . . . . . . . . . . 31

L

Labanauskas Liutauras Lanka Imants . . . . . Laugesaar Rael . . . . Lehnert Willy . . . . . . Leskauskas Darius . . Liesiene Ruta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 30 31 35 22 39

F

Fensom Anthony . . . . . . . . . . . . . . . . . . 36

G

Gelziniene Giedre . . . . . . . . . . . . . . . . . . 34 Gjergja Romana . . . . . . . . . . . . . . . . . . . 27 Gradeckiene Svajune . . . . . . . . . . . . 28, 32, 36 Grigoniene Jurate . . . . . . . . . . . . . . 27, 35-36 Grikiniene Jurgita . . . . . . . . . . . . . . . . 27, 38 Grinkeviciute Dovile . . . . . . . . . . . . . . . . . 19 Gumbeleviciene Lingvita . . . . . . . . . . . . . . 20 Guney Naile . . . . . . . . . . . . . . . . . . . . . 28 Gursoy Suleyman . . . . . . . . . . . . . . . . 31-32

M

Magistris Michel R. . . . Malenica Masa. . . . . . Mannamaa Mairi . . . . . Markvaldiene Genute . . Matukevicius Algimantas Mauricas Mykolas . . . . Mazeikaite Renata . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18 27 19 33 20 29 38

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

Mezinska Ilze . . Mikulenaite Laima Morten Enrico . . Muhle Hiltrud . .

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. . 30 18, 30 . . 26 . . 24

N

Napa Aita . . . . . Narkeviciute Irena . Nechay Alla . . . . Nielsen Hanne . . . Nikanorova Marina Norkiene Lina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 37 39 33 20 28

Siurnaite Inga . . . Skerliene Birute . . Smulska Nataliya . Sojat Tina . . . . . Spaapen Leo . . . Spokiene Indre . . Stakisaitis Donatas Starup Lene . . . . Stephani Ulrich . . Stepko Inese. . . . Stetsenko Tatyana. Strautmanis Jurgis.

. . . . . . . . . . . .

. . . . . . . . . . . .

. . . . . . . . . . . .

. . . . . . . . . . . .

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. . . . 32 28, 35-36 . . . . 31 . . . . 27 . . . . 25 . . . . 26 . . . . 26 . . . . 34 . . . . 24 . . . . 30 . . . . 39 . . 21, 39

T O

Olofsson Kern . . . . . . . . . . . . . . . . . . . . 33 Ostrauskiene Danguole . . . . . . . . . . . . . . . 28 Ozdincler Arzu Razak . . . . . . . . . . . . . . 31-32 Talvik Inga . . . . . . . . . . . . . . . . . . . . 19, 36 Talvik Tiina . . . . . . . . . . . . . . . . . . 19, 27, 36 Tarakci Ela . . . . . . . . . . . . . . . . . . . . 31-32 Taruscio Domenica . . . . . . . . . . . . . . . . . 26 Tripathi Manjiri . . . . . . . . . . . . . . . . . . . . 33 Tuleviciene Jura . . . . . . . . . . . . . . . 28, 33, 37 Tumiene Birute . . . . . . . . . . . . . . . . . . . 36 Tumsevica Zane . . . . . . . . . . . . . . . . . . . 30

P

Pavare Zane . . . . . . . . Petroska Donatas . . . . . Pilviniene Rugile . . . . . . Plioplys Sigita . . . . . . . Plouin Perrine . . . . . . . Posada de La Paz Manuel Praninskiene Ruta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32 . . 28, 35 . . . . 31 . . 21, 23 . . . . 23 . . . . 26 29, 33, 37

U

Uloziene Ingrida . Urboniene Daiva . Utkus Algirdas . . Uysal Hakan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 . . 36 . . 36 31-32

R

Rackauskaite Dovile . Ragaisis Vytautas . . Rajakrishnan V. . . . Rudnicka Svetlana. . Rutkauskiene Giedre . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27, 34 19, 39 . . 33 . . 30 20, 38

V

Vaher Ulvi . . . . . . . . . . . . . . . . . . . . . . 27 Vaiciene Nerija . . . . . . . . . . 20, 27-29, 32, 34-38 Vainauskiene Jurgita . . . . . . . . . . . . . . . . 34 van Emde Boas Walter . . . . . . . . . . . . . . . 20 Veri Kadi . . . . . . . . . . . . . . . . . . . . . . . 36 Vetra Anita . . . . . . . . . . . . . . . . . . . . 18, 32 Vinogradovs Aleksejs . . . . . . . . . . . . . . . . 30 Volkova Tatjana . . . . . . . . . . . . . . . . . . . 37 Vollmer Brigitte . . . . . . . . . . . . . . . . . . . 18 von Spiczak Sarah. . . . . . . . . . . . . . . . . . 24 Vysniauskiene Danute . . . . . . . . . . . . 33, 36-37

S

Sabol Zlatko . . . . Saferis Viktoras . . Sahlholdt Lene. . . Samaitiene Ruta . . Savendahl Lars . . Savickiene Daiva . Savlovskis Janis . . Schieppati Arrigo . Sejersen Thomas . Serapinas Danielius Silanskaite Jolita . . Simaityte Inga . . . Singh Anit . . . . . Sinkeviciene Janina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 . . 34 . . 34 18, 30 . . 26 . . 37 . . 30 . . 25 . . 25 . . 36 . . 32 . . 34 . . 33 . . 37

W

Ward Chandra . . . . . . . . . . . . . . . . . . . . 36

Z

Zaveckiene Kristina . . . . . . . . . . . . . . . . . 31 Zukauskaite Gintare . . . . . . . . . . . . . . . . . 35

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LIST OF PARTICIPANTS

9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

LIST OF PARTICIPANTS (registered up to May 1)

CROATIA

Cvitanovic Sojat Ljerka [email protected]

INDIA

Singh Anit [email protected]

ISRAEL

Basel-Vanagaite Lina [email protected] Beni-Adani Liana [email protected]

DENMARK

Alving Jorgen [email protected] Holm Anne Marie [email protected] Jaegergaard Jane [email protected] Nielsen Hanne [email protected] Nikanorova Marina [email protected] Sahlholdt Lene [email protected]

ITALY

Baiardi Paola [email protected] Schieppati Arrigo [email protected]

LATVIA

Bingeliene Arina [email protected] Cudere Anna [email protected] Jostmane Ilze [email protected] Kazaine Inese [email protected] Kikule Ilga [email protected] Kogane Jekaterina [email protected] Kondrate Kristina [email protected] Krauce Marika [email protected] Krumina Zigrida [email protected] Kupcs Karlis [email protected] Lanka Imants [email protected] Lindenberga Sarma [email protected] Madre Sarmite [email protected] Meiksane Vizma [email protected] Mezinska Ilze [email protected] Norite Baiba [email protected] Ozola Sarmite [email protected] Pavare Zane [email protected] Rozentals Guntis [email protected] Strautmanis Jurgis [email protected]

ESTONIA

Ennok Margus [email protected] Kolk Anneli [email protected] Rein Reet [email protected] Seling Ly [email protected] Siitan Maia [email protected] Tali Reet [email protected] Talvik Inga [email protected] Talvik Tina [email protected] Teeaar Anne [email protected] Vaher Ulvi [email protected]

FRANCE

Dulac Olivier [email protected] Plouin Perrine [email protected]

GERMANY

Stephani Ulrich [email protected]

GREECE

Covanis Anthanasios [email protected]

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

Tihomirova Margarita [email protected] Valge Liga [email protected] Vetra Anita [email protected] Vitols Egils [email protected] Voita Valda [email protected]

LITHUANIA

Abelyte Rasa [email protected] Adomaitiene Lione [email protected] Alaune Arvydas [email protected] Bakaniene Indre [email protected] Balkaitiene Rita [email protected] Bandanskyte Ausra [email protected] Barkauskiene Praurima [email protected] Bieliauskiene Laimute [email protected] Brusokiene Grazina [email protected] Dambrauskiene Milda [email protected] Dolebiene Ruta [email protected] Drulyte Audrone [email protected] Endziniene Milda [email protected] Gradeckiene Svajune [email protected] Grendaite Birute [email protected] Greseviciene Vitalija [email protected] Grigoniene Jurate [email protected] Grikiniene Jurgita [email protected] Grinkeviciute Dovile [email protected] Gumbeleviciene Lingvita [email protected] Jaskeviciene Vanda [email protected] Judickiene Asta [email protected] Jurgaitiene Ruta [email protected]

Jurgaityte Donata [email protected] Jurkeviciene Giedre [email protected] Kaminskiene Aurika [email protected] Kanyte Edita [email protected] Kiudeliene Rosita [email protected] Kuliesiene Ausra [email protected] Kumetaitiene Ona [email protected] Kusliene Dalia [email protected] Leskauskas Darius [email protected] Liesiene Ruta [email protected] Maciuleviciene Ruta [email protected] Mameniskiene Ruta [email protected] Markvaldiene Gene [email protected] Maskeliene Vida [email protected] Maskolenkaite Giedre [email protected] Matukevicius Algimantas [email protected] Mikulenaite Laima [email protected] Mykolaityte Diana [email protected] Niemciene Vida [email protected] Norkiene Lina [email protected] Ostrauskiene Danguole [email protected] Paulikiene Violeta [email protected] Pauza Valius [email protected] Pavalkiene Snieguole [email protected] Praninskiene Ruta [email protected] Priceviciute Renata [email protected] Rackauskaite Dovile [email protected] Ragaisis Vytautas [email protected] Rancelyte Rasa [email protected]

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9th International Conference of Baltic Child Neurology Association (BCNA) · June 20­23, 2007, Vilnius, Lithuania

Rastenyte Daiva [email protected] Roviene Daiva [email protected] Rutkauskiene Giedre [email protected] Samaitiene Ruta [email protected] Savickiene Daiva [email protected] Savickiene Danute ­ Regina [email protected] Savilovas Algimantas [email protected] Serapinas Danielius [email protected] Sereikiene Rima [email protected] Seskiene Jolanta [email protected] Simkoniene Alicija [email protected] Sinkeviciene Nijole [email protected] Skerliene Birute [email protected] Spokiene Indre [email protected] Strupiene Laimute [email protected] Tabariene Valerija [email protected] Tamasauskiene Liudmila [email protected] Utkus Algirdas [email protected] Vaiciene Nerija [email protected] Vedeckiene Dalia [email protected] Verkiene Ieva [email protected] Volkova Tatjana [email protected] Vysniauskiene Danute [email protected] Zaveckiene Kristina [email protected] Zeguniene Sigita [email protected] Zukauskaite Gintare [email protected]

SWEDEN

Ekberg Gunilla [email protected] Jucaite Aurelija [email protected] Ljungberg Ann-Christine [email protected] Olsson Ulla [email protected] Sejersen Thomas [email protected] Vollmer Brigitte [email protected]

SWITZERLAND

Boltshauser Eugen [email protected] Magistris Michel R. [email protected]

THE NETHERLANDS

Spaapen Leo [email protected] van Emde Boas Walter [email protected]

TURKEY

Cavlak Ugur [email protected] Kavlak Erdogan [email protected]

UKRAINE

Chepiga Ludmila [email protected] Davydova Iuliya [email protected] Nechay Alla [email protected] Ryslyayeva Viktoriya [email protected] Smulska Nataliya [email protected]

UNITED KINGDOM

Stephenson John BP [email protected]

USA

Brumback Roger [email protected] Caplan Rochelle [email protected] Plioplys Sigita [email protected]

SERBIA

Jovic Nebojsa [email protected]

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To the Authors of "Seminars in Neurology"

The Journal "Seminars in Neurology" presents review articles and original research papers on the latest advances in etiopathogenesis, clinics, diagnostics, treatment and prevention of neurological, neurosurgical and adjacent pathology. It publicizes, as well, case reports about rare or diagnostically complicated clinical events, materials of scientific conferences, and other important scientific information. Articles are presented in English or in Lithuanian. Articles already presented in other scientific publications are not accepted to the journal except if they had been submitted as abstracts. All the scientific articles submitted for publication are being reviewed by Editorial board and experts of particular field of medicine. Scientific articles are published in the journal complimentary. The volume of manuscript (including syllabustext, charts, pictures, images) should not exceed 30 typed pages, and the list of references should not contain more than 60 quoted sources. It is necessary to indicate personal address and e-mail of the author whom Editorial board might contact with, as well, first and last names, degrees, institutions and offices of all authors. The structure of the article: title, names of the authors; institution (-s), where the research/work has been performed; summary; keywords, syllabus-text; references. Original research paper's text and summary consists of preface, objective, methods, results, discussion; conclusions. The structure of review article is chosen by the author. It is necessary to indicate that the article is considered to be a review. The text of case report includes preface, description of the case (-s), discussion. All the abbreviations used in the text should be explained. All the charts are submitted on separate pages, numerated according their sequence in the text, include their titles. Illustrations should be compact, suitable for printed reproduction, pictures and diagrams painted with ink or computer (Corel DRAW

preferred), positives contrast, on glance paper. While submitting photo images on CD, TIFF size (distinction of at least 200 dpi) is preferred. Each illustration or image should have its title. The places where the charts (pictures, diagrams, images) are to be inserted should be marked by the author. The author's name, title of the article, numeration and the top of the image should be indicated on the other side of each illustration. List of references includes the sources of literature quoted in the text according to sequence of citation. It should be printed on a separate page in original language. The citation number of reference is indicated in square brackets.

EXAMPLE: References Article: 1. Brodie MI, Por ter RI. New and po ten tial anticonvulsants. Lancet 1990; 336: 425­6. 2. Corbo M, Nemmi R, Iannaccone S, et al. Peripheral neuropathy in scleroderma. Clin Neuropathol 1993; 12: 63­7. Chapter: 3. Biller I. Non-atherosclerotic vasculopathies. In: Biller I, ed. Stroke in children and young adults. Boston: Butterworth-Heineman, 1994; 57­81. Book: 4. Klumbys L. Ûminës galvos smegenø traumos neurofiziologija. V., 1979. 5. Pryse-Phillips W. Companion to clinical neurology. Boston: Little, Brown, 1995.

The manuscripts are submitted to the Editor-inchief V. Budrys by post or e-mail. Editorial address: Vilnius University Hospital Santariðkiø Klinikos Santariðkiø 2, LT-08661 Vilnius, Lithuania Tel./fax (370 5) 2365 220 Mob. phone +370 698 44049 e-mail: [email protected] http://www.neuro.lt

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