Clinicopathological studies are the gold standard for any medical research. These sorts of studies find brains of those with certain diseases and then refer back to the patients’ medical records to see what observations can be made and lessons learned.
This Japanese clinicopathological study looks at 22 patients with path-confirmed PSP. I think the most recent Williams/UK study looked at 85 patients.
“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.” This study “demonstrates that PSP patients manifest cerebellar ataxia.” (Dr. David Williams came up with the classification of RS or Richardson’s Syndrome and PSP-P or PSP-Parkinsonism. His research since 2005 or so describes these two main types of PSP.)
Robin
Movement Disorders. 2009 May 1. [Epub ahead of print]
Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study.
Kanazawa M, Shimohata T, Toyoshima Y, Tada M, Kakita A, Morita T, Ozawa T, Takahashi H, Nishizawa M.
Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan.
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.
PubMed ID#: 19412943 (see pubmed.gov for abstract only)