A critical review of white matter changes in Huntington’s disease

Casella, Chiara, Rosser, Anne ORCID: https://orcid.org/0000-0002-4716-4753, Lipp, Ilona, Jones, Derek ORCID: https://orcid.org/0000-0003-4409-8049 and Metzler-Baddeley, Claudia ORCID: https://orcid.org/0000-0002-8646-1144 2020. A critical review of white matter changes in Huntington’s disease. Movement Disorders 35 (8) , pp. 1302-1311. 10.1002/mds.28109

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Abstract

Huntington’s disease is a genetic neurodegenerative disorder. White matter alterations have recently been identified as a relevant pathophysiological feature of Huntington’s disease, but their etiology and role in disease pathogenesis and progression remain unclear. Increasing evidence suggests that white matter changes in this disorder are due to alterations in myelin-associated biological processes. This review first discusses evidence from neurochemical studies lending support to the ‘De-myelination hypothesis’ of Huntington’s disease, and demonstrating aberrant myelination and changes in oligodendrocytes in the Huntington’s brain. Next, evidence from neuroimaging studies is reviewed, the limitations of the described methodologies are discussed, and suggested interpretations of findings from published studies are challenged. Although our understanding of Huntington’s associated pathological changes in the brain will increasingly rely on neuroimaging techniques, the shortcomings of these methodologies must not be forgotten. Advances in MRI techniques and tissue modeling will enable a better in vivo, longitudinal characterization of the biological properties of white matter microstructure. This, in turn, will facilitate identification of disease-related biomarkers and the specification of outcome measures in clinical trials.

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Biosciences Cardiff University Brain Research Imaging Centre (CUBRIC) Medicine Psychology
Publisher:	Wiley
ISSN:	0885-3185
Funders:	Wellcome Trust
Date of First Compliant Deposit:	23 June 2020
Date of Acceptance:	30 April 2020
Last Modified:	25 Nov 2024 12:01
URI:	https://orca.cardiff.ac.uk/id/eprint/131346

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