Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study
Identifieur interne : 001604 ( Istex/Corpus ); précédent : 001603; suivant : 001605Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study
Auteurs : Masato Kanazawa ; Takayoshi Shimohata ; Yasuko Toyoshima ; Mari Tada ; Akiyoshi Kakita ; Takashi Morita ; Tetsutaro Ozawa ; Hitoshi Takahashi ; Masatoyo NishizawaSource :
- Movement Disorders [ 0885-3185 ] ; 2009-07-15.
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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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DOI: 10.1002/mds.22583
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<copyright ownership="thirdParty">Copyright © 2009 Movement Disorder Society</copyright>
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<correspondenceTo>Department of Neurology, Brain Research Institute, Niigata University, 1‐757 Asahimachi‐dori, Chuoku, Niigata, Niigata 951‐8585, Japan</correspondenceTo>
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<titleGroup><title type="main" xml:lang="en">Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study<link href="#fn1"></link>
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<title type="short" xml:lang="en">PSP: A Distinct Phenotype with Cerebellar Ataxia</title>
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<keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">progressive supranuclear palsy</keyword>
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<p>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</p>
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<noteGroup><note xml:id="fn1"><p>Potential conflict of interest: Nothing to report.</p>
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<abstract lang="en">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</abstract>
<note type="content">*Potential conflict of interest: Nothing to report.</note>
<subject lang="en"><genre>Keywords</genre>
<topic>progressive supranuclear palsy</topic>
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