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) 40 50 70 10 ADL 4 5 50% 5 6 II.

2 PSP(PSP-RS) a) PSP 2016/10 2

3 PSP 80 100% b) PSP 2016/10 3

4 PSP PSP PSP PSP FTLD PSP III. PSP(PSP-RS) a) PSP PSP - - CBD HE 2016/10 4 (CT, MRI)

5 -A -B III III SPECT PET b) PSP (NFT) pretangle tufted astrocyte coiled body argyrophilic thread NFT globose type NFT -A, 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.

6 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) PLS b) PSP PSP-P PSP PAGF PSP C i) PSP-P (PSP-parkinsonism) 2016/10 6 PSP-RS PSP-RS ii)

7 PSP-PAGF (PSP-pure akinesia with gait freezing) PSP-RS PSP-RS PSP-P iii) PSP-C (PSP wieh cerebellar ataxia) PSP-RS IV. 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.

8 1964 . 2016/10 8 MRI . A: . B: . 2016/10 9 PSP . A: globose type . B: tufted astrocyte. C: coiled body argyrophilic thread. E: PSP tufted astrocyte NFT 1 tufted astrocyte, 1 NFT, 3.