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  • Title: [Executive dysfunction in Huntington's disease].
    Author: Redondo Vergé L, Brown RG, Chacón J.
    Journal: Rev Neurol; ; 32(10):923-9. PubMed ID: 11424047.
    Abstract:
    INTRODUCTION: In the early stages of Huntington s disease (HD), there is selective vulnerability of the medium-sized spinous cells of the striate nucleus. These cells are connected with the dorsolateral prefrontal cortex, which is an area involved in cognitive processes concerning attention and executive function, forming part of one of the cortico-striatal circuits. OBJECTIVE: To analyze and interpret the executive dysfunction of a group of patients with HD at a moderately advanced stage. PATIENTS AND METHODS: We evaluated 16 patients and controls. Three clinical scales were used: the motor section of the UHDRS, the independence scale and the total functional capacity scale. For cognitive evaluation we used the study protocol of the Core Assessment Program for Intracerebral Transplantation for HD (CAPIT-HD). RESULTS: The patients with EH showed significant deterioration on the tests to evaluate cognitive function, attention and working memory. There were only two exceptions, the WEST and the direct order digit span. CONCLUSIONS: Patients with HD have executive dysfunction, probably due to dysfunction of the cortico-striatal circuits. We therefore agree with the proposal that fronto-striatal cognitive dysfunction is the most suitable name for the cognitive deterioration seen in this condition.
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