進行性核上性麻痺の 病態と治療 - snh.or.jp

1 1 PSP MRI hummingbird sign MRI MRI 1999 10 10 10 20 27 1 1 PSP-P Parkinsonism PSP- PAGF pure akinesia with gait freezing PSP- CBS / 2 3 Warren NM, Piggott MA, Greally E, et al.

2 Mov Disord 2007; 15: 1594-1600. Seppi K, Scherfler C, Donnemiller E, et al. Arch Neurol 2006; 63: 1154-1160. Pascual J, Berciano J, Gonz lez AM, et al. J Neurol Sci. 1993; 114: 165-169. Gilman S, Koeppe RA, Nan B, et al. Neurology 2010; 74: 1416-1423. Pascual, Berciano, Gonz lez, et al. J Neurol Sci. 1993 1 -51% 2 -72% 3 -58% 4-6 -65% 1 -73% 2 -74% 3 -82% 4-6 -95% CA1 60% 72% 71% 46% 38% 38% 62% 53% - 69% Warren, Piggott, Greally, et al. Movement Disorder 2007 2006 2006 2006 3 1 3 2 3 2 8 3 1 6 6 1 PSPRS-J age carbidopa/ levodopa droxidopa galantamine

3 Rivastigmine Patient 1 F 78 40/400 800 - 9 Patient 2 M 82 40/400 800 - 9 Patient 3 F 67 40/400 800 16 - Patient 4 M 73 40/400 800 16 - Patient 5 M 83 40/400 800 - 9 Patient 6 F 76 40/400 800 8 - Patient 7 F 82 40/400 800 8 - Patient 8 F 88 40/400 800 8 - (mg/day) Patient 1 Patient 2 Patient 3 Patient 6 Patient 4 Patient 8 Patient 7 Patient 5 2 PSP 8 6 MMSE HDS-R mean SD paired t test p= p< mean SD 26 27 15 17 23 27 28 28 27 28 19 26 22 19 26 28 05101520253029 29 11 11 19 22 24 27 22 25 16 24 18 22 26 28 051015202530 2 PSP 8 6 FAB mean SD p< 10 13 5 6 9 14 10 12 12 15 6 11 6 9 12 14 0246810121416 2 PSP 8 6 10m ( ) ( / Mean SD P< P< Mean SD 0510152025308090100110120130140150160 2 PSP 8 6 ( ) Timed up & go ( ) mean SD P< P< mean SD 0200400600800100005101520253035 2 PSP 8 6 1 PSPRS-J mean SD P< 10152025303540P< 3 3 2 3 3 e BMS-986168 BMS-986168 2017 4 2021 6 30 2017 10 2018 7 17 94 USA, Canada, , Australia, Austria, CZE, Germany, France, Italy, Mexico, NL, Poland, Russia, Spain, Sweden, UK.)

4 7 30 3 52 2 1 PSP Probable Possible 40 85 41 86 5 10 Mini Mental State Exam MMSE 20 43kg 120kg MRI 60 52 30 MRI 1 cm3 3 2018 7 31