Potential conflict of interest: Nothing to report.
Research Article
Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study†
Article first published online: 1 MAY 2009
DOI: 10.1002/mds.22583
Copyright © 2009 Movement Disorder Society
Additional Information
How to Cite
Kanazawa, M., Shimohata, T., Toyoshima, Y., Tada, M., Kakita, A., Morita, T., Ozawa, T., Takahashi, H. and Nishizawa, M. (2009), Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study. Mov. Disord., 24: 1312–1318. doi: 10.1002/mds.22583
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Publication History
- Issue published online: 24 JUL 2009
- Article first published online: 1 MAY 2009
- Manuscript Accepted: 9 MAR 2009
- Manuscript Revised: 25 DEC 2008
- Manuscript Received: 24 SEP 2008
- Abstract
- Article
- References
- Cited By
Keywords:
- progressive supranuclear palsy;
- spinocerebellar ataxias;
- Parkinson's disease;
- parkinsonian disorders
Abstract
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. © 2009 Movement Disorder Society

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