|Title:||Cerebellar Pathology in Early Onset and Late Onset Essential Tremor||Authors:||Kuo S.-H.
|Keywords:||Age of onset; Cerebellum; Essential tremor; Neurodegenerative; Pathology; Purkinje cell||Issue Date:||2017||Journal Volume:||16||Journal Issue:||2||Start page/Pages:||473-482||Source:||Cerebellum||Abstract:||
Early onset and late onset essential tremor (ET) cases differ in several respects. Whether they differ with respect to cerebellar pathologic changes remains to be determined. We quantified a broad range of postmortem features (Purkinje cell (PC) counts, PC axonal torpedoes and associated axonal changes, heterotopic PCs, and hairy basket ratings) in 30 ET cases with age of tremor onset <50?years, 30 ET cases with age of tremor onset ?50?years, and 30 controls (total n?=?90). We also used two alternative age of onset cut-points (<40 vs. ?40?years, and <60 vs. ?60?years) to define early onset vs. late onset ET. We found that ET cases with tremor onset <50?years and tremor onset ?50?years had similar PC counts (8.78?±?1.70 vs. 8.86?±?1.24, p?=?0.839), PC axonal torpedo counts (17.87?±?18.27 [median =13.00] vs. 12.90?±?10.60 [median =9.0], p?=?0.486) and associated axonal pathology (all p values >0.05), heterotopic PC counts (9.90?±?11.55 [median =6.00] vs. 5.40?±?5.10 [median =3.50], p?=?0.092), and hairy basket ratings (1.95?±?0.62 [median =2.00] vs. 2.05?±?0.92 [median =2.00], p?=?0.314). When using the age of onset cut-points of 40 or 60?years, results were similar. Early onset and late onset ET cases share similar cerebellar postmortem features. These data do not support the notion that these age-of-onset related forms of ET represent distinct clinical-pathological entities. ? 2016, Springer Science+Business Media New York.
|URI:||https://scholars.lib.ntu.edu.tw/handle/123456789/506166||ISSN:||1473-4222||DOI:||10.1007/s12311-016-0826-5||SDG/Keyword:||adult; Article; axon; cell count; cerebellum cortex; cerebellum disease; clinical feature; controlled study; disease duration; essential tremor; female; human; human cell; major clinical study; male; onset age; postmortem change; Purkinje cell; cerebellum; comparative study; essential tremor; onset age; pathology; prospective study; very elderly; Age of Onset; Aged, 80 and over; Cerebellum; Essential Tremor; Female; Humans; Male; Prospective Studies
|Appears in Collections:||藥理學科所|
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