Unusual clinical presentations of cortical‐basal ganglionic degeneration
Identifieur interne : 004484 ( Main/Merge ); précédent : 004483; suivant : 004485Unusual clinical presentations of cortical‐basal ganglionic degeneration
Auteurs : C. Bergeron [Canada] ; M. S. Pollanen ; L. Weyer ; S. E. Black [Canada] ; A. E. LangSource :
- Annals of Neurology [ 0364-5134 ] ; 1996-12.
Abstract
Cortical‐basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution, swollen achromasic neurons, and extensive deposition of abnormal tau in neurons and glia. We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms. In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe, amygdala, and hippocampus. In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical‐basal ganglionic degeneration, the most severe changes were observed in the left motor cortex and adjacent Broca's area. It is therefore apparent that the histological changes of cortical‐basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions. On this basis we conclude that cortical‐ basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia, particularly when the latter is accompanied by frontal lobe symptomatology, early speech alterations, or parkinsonism.
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DOI: 10.1002/ana.410400611
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Bergeron</name><affiliation></affiliation><affiliation></affiliation><affiliation></affiliation><affiliation wicri:level="4"><country xml:lang="fr">Canada</country><wicri:regionArea>Centre for Research in Neurodegenerative Diseases, University of Toronto, 6 Queen's Park West, Toronto, M5S 3H2 Ontario</wicri:regionArea><orgName type="university">Université de Toronto</orgName><placeName><settlement type="city">Toronto</settlement><region type="state">Ontario</region></placeName></affiliation></author><author><name sortKey="Pollanen, M S" sort="Pollanen, M S" uniqKey="Pollanen M" first="M. S." last="Pollanen">M. S. Pollanen</name><affiliation><wicri:noCountry code="subField">Toronto</wicri:noCountry></affiliation></author><author><name sortKey="Weyer, L" sort="Weyer, L" uniqKey="Weyer L" first="L." last="Weyer">L. 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Lang</name><affiliation></affiliation><affiliation></affiliation><affiliation><wicri:noCountry code="subField">Toronto</wicri:noCountry></affiliation></author></analytic><monogr></monogr><series><title level="j">Annals of Neurology</title><title level="j" type="sub">Official Journal of the American Neurological Association and the Child Neurology Society</title><title level="j" type="abbrev">Ann Neurol.</title><idno type="ISSN">0364-5134</idno><idno type="eISSN">1531-8249</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="1996-12">1996-12</date><biblScope unit="volume">40</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="893">893</biblScope><biblScope unit="page" to="900">900</biblScope></imprint><idno type="ISSN">0364-5134</idno></series><idno type="istex">4FC5057538C8A8E6F4777DAA9CCF08FB385F2111</idno><idno type="DOI">10.1002/ana.410400611</idno><idno type="ArticleID">ANA410400611</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0364-5134</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Cortical‐basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution, swollen achromasic neurons, and extensive deposition of abnormal tau in neurons and glia. We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms. In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe, amygdala, and hippocampus. In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical‐basal ganglionic degeneration, the most severe changes were observed in the left motor cortex and adjacent Broca's area. It is therefore apparent that the histological changes of cortical‐basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions. On this basis we conclude that cortical‐ basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia, particularly when the latter is accompanied by frontal lobe symptomatology, early speech alterations, or parkinsonism.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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