Cerebellar degeneration correlates with motor symptoms in Huntington’s disease

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dc.contributor.author Singh-Bains, Malvindar en
dc.contributor.author Mehrabi, Fahimeh en
dc.contributor.author Sehji, T en
dc.contributor.author Austria, Micah Daniel en
dc.contributor.author Tan, Adelie en
dc.contributor.author Tippett, Lynette en
dc.contributor.author Dragunow, Michael en
dc.contributor.author Waldvogel, Henry en
dc.contributor.author Faull, Richard en
dc.date.accessioned 2019-03-29T03:14:23Z en
dc.date.issued 2019-03 en
dc.identifier.citation Annals of neurology 85(3):396-405 Mar 2019 en
dc.identifier.issn 0364-5134 en
dc.identifier.uri http://hdl.handle.net/2292/46327 en
dc.description.abstract Objective Huntington disease (HD) is an autosomal dominant neurodegenerative disorder characterized by variable motor and behavioral symptoms attributed to major neuropathology of mainly the basal ganglia and cerebral cortex. The role of the cerebellum, a brain region involved in the coordination of movements, in HD neuropathology has been controversial. This study utilizes postmortem human brain tissue to investigate whether Purkinje cell degeneration in the neocerebellum is present in HD, and how this relates to disease symptom profiles. Methods Unbiased stereological counting methods were used to quantify the total number of Purkinje cells in 15 HD cases and 8 neurologically normal control cases. Based on their predominant symptoms, the HD cases were categorized into 2 groups: “motor” or “mood.” Results The results demonstrated a significant 43% loss of Purkinje cells in HD cases with predominantly motor symptoms, and no cell loss in cases showing a major mood phenotype. There was no significant correlation between Purkinje cell loss and striatal neuropathological grade, postmortem delay, CAG repeat in the IT15 gene, or age at death. Interpretation This study shows a compelling relationship between Purkinje cell loss in the HD neocerebellum and the HD motor symptom phenotype, which, together with our previous human brain studies on the same HD cases, provides novel perspectives interrelating and correlating the variable cerebellar, basal ganglia, and neocortical neuropathology with the variability of motor/mood symptom profiles in the human HD brain. en
dc.publisher Wiley en
dc.relation.ispartofseries Annals of Neurology en
dc.rights Items in ResearchSpace are protected by copyright, with all rights reserved, unless otherwise indicated. Previously published items are made available in accordance with the copyright policy of the publisher. en
dc.rights.uri https://researchspace.auckland.ac.nz/docs/uoa-docs/rights.htm en
dc.rights.uri https://creativecommons.org/licenses/by-nc/4.0/ en
dc.title Cerebellar degeneration correlates with motor symptoms in Huntington’s disease en
dc.type Journal Article en
dc.identifier.doi 10.1002/ana.25413 en
pubs.issue 3 en
pubs.begin-page 396 en
pubs.volume 85 en
dc.rights.holder Copyright: The authors en
pubs.end-page 405 en
dc.rights.accessrights http://purl.org/eprint/accessRights/OpenAccess en
pubs.subtype Article en
pubs.elements-id 760928 en
pubs.org-id Medical and Health Sciences en
pubs.org-id Medical Sciences en
pubs.org-id Anatomy and Medical Imaging en
pubs.org-id Pharmacology en
pubs.org-id Science en
pubs.org-id Psychology en
pubs.record-created-at-source-date 2019-02-05 en
pubs.online-publication-date 2019-01-11 en
pubs.dimensions-id 30635944 en

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