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Actas Esp Psiquiatr. 2010 Sep-Oct;38(5):285-94. Epub 2010 Sep 1.

Juvenile Huntington's disease: a case report and literature review.

[Article in English, Spanish]

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  • 1Instituto de Psiquiatría y Salud Mental, Hospital Clínico San Carlos, Madrid, España. laura_reyes_molon@yahoo.es

Abstract

Huntington's disease is the most frequent neurodegenerative disease with a prevalence of fewer than 10 cases per 10,000 inhabitants; the juvenile form is responsible for less than 10% of all cases. Huntington's disease belongs to the group known as "triad syndromes," which evolve with cognitive, motor and neuropsychiatric manifestations. Around 30% of patients debut with behavioral symptoms, which are a major challenge for management by patients, families, and caregivers. Huntington's disease (HD) is reviewed and a case of juvenile onset is reported in this article. The characteristics of juvenile-onset Huntington's disease (HD) differ from those of adult-onset HD, as chorea does not occur, although bradykinesia, dystonia, and signs of cerebellar disorder, such as rigidity, are present, frequently in association with convulsive episodes and psychotic manifestations.

PMID:
21117003
[PubMed - indexed for MEDLINE]
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