Neuropsychiatric View of Huntington‘s Disease


Authors: J. Necpál 1;  M. Patarák 2
Authors‘ workplace: Neurologické oddelenie, Nemocnica Zvolen a. s. 1;  Psychiatrické oddelenie, FNsP F. D. Roosevelta Banská Bystrica 2
Published in: Cesk Slov Neurol N 2013; 76/109(4): 438-445
Category: Review Article

Overview

Huntington’s disease is a progressive and fatal neurodegenerative disorder with an incidence of about 5 cases per 100,000 people. There is familial occurrence, with autosomal dominant inheritance. The more family members affected, the greater the burden these families have. A typical phenotype is choreatic hyperkinesia, though rigidity, hypokinesia and gait disturbance appear years later. Cognitive and psychiatric symptoms are typical, often pre­ceding clinical expression of movement disorders. From the clearly defined genotype, a trio of pathological symptoms grows, with considerable phenomenological polymorphic variations from individual to individual. Psychiatric disorders often impair the quality of life more than motor abnormalities and can lead to suicide, the second leading cause of death in individuals with Huntington’s disease. Therefore, the neuropsychiatric view of this disease is not just a quantitative synthesis of findings from neurology and psychiatry, but rather an integral and comprehensive approach is also taken, allowing both a better understanding of the pathogenesis and a mostly superior therapeutic approach that takes into account se­veral aspects of the multifaceted suffering endured by these patients. The article presents an overview of the theory from a neuropsychiatric perspective on Huntington’s disease, as well as two short case studies of patients – siblings who have the disease.

Key words:
Huntington’s disease – chorea – depression –suicide – dementia – executive dysfunction


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Paediatric neurology Neurosurgery Neurology

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