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Language Disorders in Huntington Disease

A Systematic Literature Review

Gagnon, Maude, MSc*; Barrette, Jasmine, MSc*; Macoir, Joël, PhD*,†

Cognitive and Behavioral Neurology: December 2018 - Volume 31 - Issue 4 - p 179–192
doi: 10.1097/WNN.0000000000000171
Review Article
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Objective: A systematic review examining the presence and nature of language disorders associated with Huntington disease (HD).

Background: HD is characterized by gradual motor dysfunction, psychiatric problems, and cognitive decline. Communication abilities in HD may be affected not only by dysarthria but also by language impairment. However, the nature and type of this impairment is not well defined.

Methods: We searched the PubMed and PsycINFO databases and selected studies on the basis of the original language of the article, peer-review status, and specificity of the results regarding language and communication disorders.

Results: Thirty-one articles meeting the selection criteria were selected for this review. According to most of the studies, individuals with HD present with primary deficits of language. However, a few authors suggested that language deficits in HD result from nonlinguistic impairments, or that language abilities are largely preserved. More specifically, studies showed that HD is associated with difficulties in producing and understanding sentences and discourse, processing semantic representations of object and action concepts, retrieving lexical forms of nouns, and applying morphological and syntactic rules. There is some disagreement regarding whether HD affects reading abilities, sentence production, semantic processing, and application of morphological rules in verb conjugation.

Conclusions: Although people with HD present with language impairment, further studies are needed to identify their functional origin. Clinical studies are also needed to determine the impact of such impairments on an individual’s functional communication in daily living and to chart the progression of the impairments over the course of the disease.

*Speech-Language Pathology Program, Department of Rehabilitation, Faculty of Medicine, Laval University, Quebec City, Quebec, Canada

CERVO Brain Research Centre, Mental Health University Institute of Quebec, Quebec City, Quebec, Canada

The authors declare no conflicts of interest.

Correspondence: Joël Macoir, PhD, Department of Rehabilitation, Faculty of Medicine, Laval University, Pavillon F-Vandry, Quebec City, Quebec, Canada G1K 7P4 (e-mail: joel.macoir@fmed.ulaval.ca).

Received December 19, 2017

Accepted September 14, 2018

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