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[] Progressive Surpranuclear PalsyPSP ,(1)PSP NINDS PSP (2)PSP 3 (3) SPECT (4) PSP MRI SPECT [] PSP 8 76.4 8 age-match 16 MRISPECT 74.0 6 10 MRI 1.5T TR: 8000TE: 3.22 3D-MRI DrView 12mm 9 SPMVSRAD 3D-SSP 123I-IMP SPECT Z-score [] 1 PSP patients' profile

82 5 6 4 3 1 1 () 22 13 2.31/2.08 () 29 17 8 2.23/1.90 () () 27 16 3 2.79/2.82 8 83 PSP-RS 69 PSP-P 66 6 () () 21 12 2.65/2.85 83 6 () 14 7 1.90/1.79 86 6 () 26 12 1.79/1.48 Pure Akinesia 64 1 10 () () () () () () () 26 16 9 2.30/2.49

retro pulsion () small voice tremor MMSE 29 FAB O-SIT 7 MIBG 2.42/2.46 L-DOPA response ()

Neuroimaging data MRI: 3 SPECT: MRI: 3 SPECT: MRI: 3 SPECT:

MRI: 3 SPECT: MRI: 3 SPECT: MRI: 3 SPECT: MRI: 3 SPECT: MRI:SPECT: 1

[] 8 pure-akinesia 7 3 PSP-RSPSP-P MRI SPECT 5 8 PSP pure-akinesia PSP MIBG (5) PSP PSP-P PSP- 2 PSP-RS Pure-akinesia PSP-P [] (1) Steele JCRichardson JCOlszewski JProgressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuclear dystonia and dementia. Arch Neurol 10 :333-359, 1964. (2) Williams DR et al:Characteristics of two distinct clinical phenotypes in pathologically proven progressive supra nuclear palsy:Richardson's syndrome and PSP-parkinsonism. Brain 128:1247-1258, 2005. (3) Asato R et al: Magnetic resonance imaging distinguishes progressive supranuclear palsy from multiple system atrophy. J Neural Transm 107 : 1427-1436,2000. (4) Frisoni GB et al : Brain atrophy in frontemporal dementia. J Neurol Neurosurg Psychiatry 61 : 157-165,1996. (5) /: (PSPRS) 73:573-585,2010.

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Microsoft Word - _SugitaSt_rev1