Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study.
The clinical heterogeneity of progressive supranuclear palsy (PSP), which is classified as classic Richardson's syndrome (RS) and PSP-Parkinsonism (PSP-P), has been previously discussed. We retrospectively analyzed 22 consecutive Japanese patients with pathologically proven PSP to investigate the clinicopathological heterogeneity. We investigated the clinical features both early in and at any time during the disease course. The pathological severities of neuronal loss with gliosis and tau pathology were also evaluated. On the basis of the clinical features, 10 patients were categorized as having RS, and 8 were categorized as having PSP-P. Four patients presenting with cerebellar ataxia or cerebral cortical signs were categorized as having unclassifiable PSP. Among them, 3 developed cerebellar ataxia as the initial and principal symptom. Notably, tau-positive inclusion bodies in Purkinje cells were significantly more frequently observed in the patients with cerebellar ataxia than in those without cerebellar ataxia. All the patients with cerebellar ataxia exhibited more neuronal loss with gliosis and higher densities of coiled bodies in the cerebellar dentate nucleus than those without cerebellar ataxia. This study confirms the wide spectrum of clinicopathological manifestations associated with PSP regardless of different ethnic origin, and demonstrates that PSP patients manifest cerebellar ataxia.
Department of Neurology, Brain Research Institute, Niigata University, Japan.
Masato
M
Kanazawa
Takayoshi
T
Shimohata
Yasuko
Y
Toyoshima
Mari
M
Tada
Akiyoshi
A
Kakita
Takashi
T
Morita
Tetsutaro
T
Ozawa
Hitoshi
H
Takahashi
Masatoyo
M
Nishizawa
D000368:Aged; D000369:Aged, 80 and over; D016923:Cell Death / Q000502:physiology; D002531:Cerebellum / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D009419:Nerve Tissue Proteins / Q000378:metabolism; D009474:Neurons / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D012189:Retrospective Studies; D016622:Silver Staining / Q000379:methods; D013494:Supranuclear Palsy, Progressive / Q000145:classification / Q000473:pathology* / Q000503:physiopathology; D016875:tau Proteins / Q000378:metabolism
D009419:Nerve Tissue Proteins; D016875:tau Proteins