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Language deficits in pre-symptomatic Huntington's disease: Evidence from Hungarian

Lookup NU author(s): Dr Cristina Dye

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Abstract

A limited number of studies have investigated language in Huntington's disease (HD). These have generally reported abnormalities in rule-governed (grammatical) aspects of language, in both syntax and morphology. Several studies of verbal inflectional morphology in English and French have reported evidence of over-active rule processing, such as over-suffixation errors (e.g., walkeded) and over-regularizations (e.g., digged). Here we extend the investigation to noun inflection in Hungarian, a Finno-Ugric agglutinative language with complex morphology, and to genetically proven pre-symptomatic Huntington's disease (pre-HD). Although individuals with pre-HD have no clinical, motor or cognitive symptoms, the underlying pathology may already have begun, and thus sensitive behavioral measures might reveal already-present impairments. Indeed, in a Hungarian morphology production task, pre-HD patients made both over-suffixation and over-regularization errors. The findings suggest the generality of over-active rule processing in both HD and pre-HD, across languages from different families with different morphological systems, and for both verbal and noun inflection. Because the neuropathology in pre-HD appears to be largely restricted to the caudate nucleus and related structures, the findings further implicate these structures in language, and in rule-processing in particular. Finally, the need for effective treatments in HD, which will likely depend in part on the ability to sensitively measure early changes in the disease, suggests the possibility that inflectional morphology, and perhaps other language measures, may provide useful diagnostic, tracking, and therapeutic tools for assessing and treating early degeneration in pre-HD and HD. (C) 2012 Elsevier Inc. All rights reserved.


Publication metadata

Author(s): Nemeth D, Dye CD, Sefcsik T, Janacsek K, Turi Z, Londe Z, Klivenyi P, Kincses ZT, Szabó N, Vecsei L, Ullman MT

Publication type: Article

Publication status: Published

Journal: Brain and Language

Year: 2012

Volume: 121

Issue: 3

Pages: 248-253

Print publication date: 24/04/2012

ISSN (print): 0093-934X

ISSN (electronic): 1090-2155

Publisher: Academic Press

URL: http://dx.doi.org/10.1016/j.bandl.2012.04.001

DOI: 10.1016/j.bandl.2012.04.001


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Funding

Funder referenceFunder name
Hungarian Academy of Sciences
Bolyai Scholarship
CZ.1.05/1.1.00/02.0123European Regional Development Fund
K 82068Hungarian National Research Fund (OTKA)
R01 HD049347NIH
R01 MH58189NIH

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