Next Article in Journal
Structural and Functional Neuroimaging of Visual Hallucinations in Lewy Body Disease: A Systematic Literature Review
Next Article in Special Issue
Protein Misfolding and Aggregation as a Therapeutic Target for Polyglutamine Diseases
Previous Article in Journal
Evidences from Rewarding System, FRN and P300 Effect in Internet-Addiction in Young People
Previous Article in Special Issue
Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity
Article Menu

Export Article

Open AccessReview
Brain Sci. 2017, 7(7), 83; doi:10.3390/brainsci7070083

Cognitive Changes in the Spinocerebellar Ataxias Due to Expanded Polyglutamine Tracts: A Survey of the Literature

Department of Medicine (Neuroscience), Monash University (Alfred Hospital Campus), Commercial Road, Melbourne, VIC 3004, Australia
*
Author to whom correspondence should be addressed.
Academic Editor: Edamakanti Chandrakanth Reddy
Received: 11 May 2017 / Revised: 27 June 2017 / Accepted: 6 July 2017 / Published: 14 July 2017
(This article belongs to the Special Issue Polyglutamine (PolyQ) Disorders)
View Full-Text   |   Download PDF [257 KB, uploaded 14 July 2017]

Abstract

The dominantly-inherited ataxias characterised by expanded polyglutamine tracts—spinocere bellar ataxias (SCAs) 1, 2, 3, 6, 7, 17, dentatorubral pallidoluysian atrophy (DRPLA) and, in part, SCA 8—have all been shown to result in various degrees of cognitive impairment. We survey the literature on the cognitive consequences of each disorder, attempting correlation with their published neuropathological, magnetic resonance imaging (MRI) and clinical features. We suggest several psychometric instruments for assessment of executive function, whose results are unlikely to be confounded by visual, articulatory or upper limb motor difficulties. Finally, and with acknowledgement of the inadequacies of the literature to date, we advance a tentative classification of these disorders into three groups, based on the reported severity of their cognitive impairments, and correlated with their neuropathological topography and MRI findings: group 1—SCAs 6 and 8—mild dysexecutive syndrome based on disruption of cerebello-cortical circuitry; group 2—SCAs 1, 2, 3, and 7—more extensive deficits based largely on disruption of striatocortical in addition to cerebello-cerebral circuitry; and group 3—SCA 17 and DRPLA—in which cognitive impairment severe enough to cause a dementia syndrome is a frequent feature. View Full-Text
Keywords: spinocerebellar ataxias; dentatorubral pallidoluysian atrophy; expanded polyglutamine tracts; cognition; neuropsychological changes spinocerebellar ataxias; dentatorubral pallidoluysian atrophy; expanded polyglutamine tracts; cognition; neuropsychological changes
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

Scifeed alert for new publications

Never miss any articles matching your research from any publisher
  • Get alerts for new papers matching your research
  • Find out the new papers from selected authors
  • Updated daily for 49'000+ journals and 6000+ publishers
  • Define your Scifeed now

SciFeed Share & Cite This Article

MDPI and ACS Style

Lindsay, E.; Storey, E. Cognitive Changes in the Spinocerebellar Ataxias Due to Expanded Polyglutamine Tracts: A Survey of the Literature. Brain Sci. 2017, 7, 83.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics

1

Comments

[Return to top]
Brain Sci. EISSN 2076-3425 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top