進行性核上性麻痺 progressive supranuclear palsy: PSP

1 2016/10 1 progressive supranuclear palsy: PSP I. PSP 1964 Steele PSP Richardson syndrome PSP (PSP Richardson syndrome: PSP-RS) PSP PSP-RS PSP-with frontotemporal dementia PSP-corticobasal syndrome PSP- progressive non-fluent aphasia PSP-with primary lateral sclerosis PSP-parkinsonism PSP-pure akinesia with gait freezing PSP with cerebellar ataxia PSP PSP CBD PSP CBD (FTLD)

2 40 50 70 10 ADL 4 5 50% 5 6 II. PSP(PSP-RS) a) PSP 2016/10 2

3 PSP 80 100% b) PSP

4 2016/10 3 PSP PSP PSP PSP FTLD PSP III.

5 PSP(PSP-RS) a) PSP PSP - - CBD HE

6 2016/10 4 (CT, MRI) -A -B III III SPECT PET b) PSP (NFT) pretangle tufted astrocyte coiled body argyrophilic thread NFT globose type NFT -A.

7 D Pretangle CBD tufted astrocyte -B NFT -D Tufted astrocyte PSP tufted astrocyte coiled body argyrophilic thread -C PSP CBD PSP CBD 64kDa 69kDa C PSP 33kDa CBD 37kDa tufted astrocyte astrocytic plaque C 33kDa 37kDa CBD PSP C PSP CBD III.

8 PSP PSP PSP (PSP-RS) PSP PSP-RS 50 60% PSP-P 2016/10 5 30% PSP PSP-FTD 4% PSP CBD 3% a) PSP-RS PSP-FTD PSP-PNFA PSP CBD PSP-PLS i) PSP-FTD (PSP-with frontotemporal dementia) (FTD) PSP PSP-RS PSP-FTD PSP-RS ii) PSP-CBS (PSP-corticobasal syndrome) CBD PSP-RS iii) PSP-PNFA (PSP- progressive non-fluent aphasia) PNFA PSP CBD PSP-PNFA PSP-RS iv) PSP-PLS (PSP-with primary lateral sclerosis)

9 PLS b) PSP PSP-P PSP PAGF PSP C i) PSP-P (PSP-parkinsonism) 2016/10 6 PSP-RS PSP-RS ii) PSP-PAGF (PSP-pure akinesia with gait freezing) PSP-RS PSP-RS PSP-P iii) PSP-C (PSP wieh cerebellar ataxia) PSP-RS IV.

10 NINDS diagnostic criteria NFT NFT NFT neuropil thread NFT neuropil thread tufted astrocyte tufted astrocyte glial cytoplasmic inclusion PSP CBD FTLD 2016/10 7 PSP Steele.