Cerebellar Involvement in Progressive Supranuclear Palsy: A Clinicopathological Study
Identifieur interne : 003053 ( Main/Merge ); précédent : 003052; suivant : 003054Cerebellar Involvement in Progressive Supranuclear Palsy: A Clinicopathological Study
Auteurs : Masato Kanazawa [Japon] ; Takayoshi Shimohata [Japon] ; Yasuko Toyoshima [Japon] ; Mari Tada [Japon] ; Akiyoshi Kakita [Japon] ; Takashi Morita [Japon] ; Tetsutaro Ozawa [Japon] ; Hitoshi Takahashi [Japon] ; Masatoyo Nishizawa [Japon]Source :
- Movement disorders [ 0885-3185 ] ; 2009.
Descripteurs français
- Pascal (Inist)
English descriptors
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.
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Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>5 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Morita, Takashi" sort="Morita, Takashi" uniqKey="Morita T" first="Takashi" last="Morita">Takashi Morita</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Pathology, Shinrakuen Hospital</s1><s2>Niigata</s2><s3>JPN</s3><sZ>6 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Ozawa, Tetsutaro" sort="Ozawa, Tetsutaro" uniqKey="Ozawa T" first="Tetsutaro" last="Ozawa">Tetsutaro Ozawa</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>7 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Takahashi, Hitoshi" sort="Takahashi, Hitoshi" uniqKey="Takahashi H" first="Hitoshi" last="Takahashi">Hitoshi Takahashi</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Pathology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>3 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Nishizawa, Masatoyo" sort="Nishizawa, Masatoyo" uniqKey="Nishizawa M" first="Masatoyo" last="Nishizawa">Masatoyo Nishizawa</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>7 aut.</sZ><sZ>9 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wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>7 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Shimohata, Takayoshi" sort="Shimohata, Takayoshi" uniqKey="Shimohata T" first="Takayoshi" last="Shimohata">Takayoshi Shimohata</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>7 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Toyoshima, Yasuko" sort="Toyoshima, Yasuko" uniqKey="Toyoshima Y" first="Yasuko" last="Toyoshima">Yasuko Toyoshima</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Pathology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>3 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Tada, Mari" sort="Tada, Mari" uniqKey="Tada M" first="Mari" last="Tada">Mari Tada</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>7 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Pathology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>3 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Kakita, Akiyoshi" sort="Kakita, Akiyoshi" uniqKey="Kakita A" first="Akiyoshi" last="Kakita">Akiyoshi Kakita</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Pathology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>3 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Pathological Neuroscience, Resource Branch for Brain Disease Research CBBR, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>5 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Morita, Takashi" sort="Morita, Takashi" uniqKey="Morita T" first="Takashi" last="Morita">Takashi Morita</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Pathology, Shinrakuen Hospital</s1><s2>Niigata</s2><s3>JPN</s3><sZ>6 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Ozawa, Tetsutaro" sort="Ozawa, Tetsutaro" uniqKey="Ozawa T" first="Tetsutaro" last="Ozawa">Tetsutaro Ozawa</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>7 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Takahashi, Hitoshi" sort="Takahashi, Hitoshi" uniqKey="Takahashi H" first="Hitoshi" last="Takahashi">Hitoshi Takahashi</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Pathology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>3 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author><author><name sortKey="Nishizawa, Masatoyo" sort="Nishizawa, Masatoyo" uniqKey="Nishizawa M" first="Masatoyo" last="Nishizawa">Masatoyo Nishizawa</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Brain Research Institute, Niigata University</s1><s2>Niigata</s2><s3>JPN</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>7 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Japon</country><wicri:noRegion>Niigata</wicri:noRegion></affiliation></author></analytic><series><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno><imprint><date when="2009">2009</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Cerebellum</term><term>Nervous system diseases</term><term>Parkinson disease</term><term>Spinocerebellar ataxia</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Maladie de Parkinson</term><term>Pathologie du système nerveux</term><term>Cervelet</term><term>Ataxie spinocérébelleuse</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml: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.</div></front></TEI><affiliations><list><country><li>Japon</li></country></list><tree><country name="Japon"><noRegion><name sortKey="Kanazawa, Masato" sort="Kanazawa, Masato" uniqKey="Kanazawa M" first="Masato" last="Kanazawa">Masato Kanazawa</name></noRegion><name sortKey="Kakita, Akiyoshi" sort="Kakita, Akiyoshi" uniqKey="Kakita A" first="Akiyoshi" last="Kakita">Akiyoshi Kakita</name><name sortKey="Kakita, Akiyoshi" sort="Kakita, Akiyoshi" uniqKey="Kakita A" first="Akiyoshi" last="Kakita">Akiyoshi Kakita</name><name sortKey="Morita, Takashi" sort="Morita, Takashi" uniqKey="Morita T" first="Takashi" last="Morita">Takashi Morita</name><name sortKey="Nishizawa, Masatoyo" sort="Nishizawa, Masatoyo" uniqKey="Nishizawa M" first="Masatoyo" last="Nishizawa">Masatoyo Nishizawa</name><name sortKey="Ozawa, Tetsutaro" sort="Ozawa, Tetsutaro" uniqKey="Ozawa T" first="Tetsutaro" last="Ozawa">Tetsutaro Ozawa</name><name sortKey="Shimohata, Takayoshi" sort="Shimohata, Takayoshi" uniqKey="Shimohata T" first="Takayoshi" last="Shimohata">Takayoshi Shimohata</name><name sortKey="Tada, Mari" sort="Tada, Mari" uniqKey="Tada M" first="Mari" last="Tada">Mari Tada</name><name sortKey="Tada, Mari" sort="Tada, Mari" uniqKey="Tada M" first="Mari" last="Tada">Mari Tada</name><name sortKey="Takahashi, Hitoshi" sort="Takahashi, Hitoshi" uniqKey="Takahashi H" first="Hitoshi" last="Takahashi">Hitoshi Takahashi</name><name sortKey="Toyoshima, Yasuko" sort="Toyoshima, Yasuko" uniqKey="Toyoshima Y" first="Yasuko" last="Toyoshima">Yasuko Toyoshima</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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