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Brain Advance Access published online on March 23, 2005
Brain, doi:10.1093/brain/awh472
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© The Author (2005). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org
Received February 20, 2004
Revised September 4, 2004
Accepted February 1, 2005

Article

The role of the striatum in rule application: the model of Huntington's disease at early stage

Marc Teichmann 1, Emmanuel Dupoux 2, Sid Kouider 2, Pierre Brugières 2, Marie-Françoise Boissé 3, Sophie Baudic 4, Pierre Cesaro 3, Marc Peschanski 4, and Anne-Catherine Bachoud-Lévi 5*

1 INSERM U421, IM3/Paris XII, Créteil, France; Laboratoire de Sciences Cognitives et Psycholinguistique, UMR8554, EHESS-ENS-CNRS, Paris, France
2 Laboratoire de Sciences Cognitives et Psycholinguistique, UMR8554, EHESS-ENS-CNRS, Paris, France
3 Department of Clinical Neurosciences, Henri Mondor Hospital, AP/HP, Créteil, France; ENS, Paris, France
4 INSERM U421, IM3/Paris XII, Créteil, France
5 INSERM U421, IM3/Paris XII, Créteil, France; Department of Clinical Neurosciences, Henri Mondor Hospital, AP/HP, Créteil, France; ENS, Paris, France; Departement d'Etudes Cognitives, Ecole Normale Supérieure (ENS), Paris, France

* To whom correspondence should be addressed.
Anne-Catherine Bachoud-Lévi, E-mail: bachoud{at}lscp.ehess.fr


  Abstract

Summary The role of the basal ganglia, and more specifically of the striatum, in language is still debated. Recent studies have proposed that linguistic abilities involve two distinct types of processes: the retrieving of stored information, implicating temporal lobe areas, and the application of combinatorial rules, implicating fronto-striatal circuits. Studies of patients with focal lesions and neurodegenerative diseases have suggested a role for the striatum in morphological rule application, but functional imaging studies found that the left caudate was involved in syntactic processing and not morphological processing. In the present study, we tested the view that the basal ganglia are involved in rule application and not in lexical retrieving in a model of striatal dysfunction, namely Huntington's disease at early stages. We assessed the rule-lexicon dichotomy in the linguistic domain with morphology (conjugation of non-verbs and verbs) and syntax (sentence comprehension) and in a non-linguistic domain with arithmetic operations (subtraction and multiplication). Thirty Huntington's disease patients (15 at stage I and 15 at stage II) and 20 controls matched for their age and cultural level were included in this study. Huntington's disease patients were also assessed using the Unified Huntington's Disease Rating Scale (UHDRS) and MRI. We found that early Huntington's disease patients were impaired in rule application in the linguistic and non-linguistic domains (morphology, syntax and subtraction), whereas they were broadly spared with lexical processing. The pattern of performance was similar in patients at stage I and stage II, except that stage II patients were more impaired in all tasks assessing rules and had in addition a very slight impairment in the lexical condition of conjugation. Finally, syntactic rule abilities correlated with all markers of the disease evolution including bicaudate ratio and performance in executive function, whereas there was no correlation with arithmetic and morphological abilities. Together, this suggests that the striatum is involved in rule processing more than in lexical processing and that it extends to linguistic and non-linguistic domains. These results are discussed in terms of domain-specific versus domain-general processes of rule application.

Keywords: striatum; Huntington disease; language; rule application; lexical retrieving.
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