Juvenile Huntington's disease: a case report and literature review.
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.
Instituto de Psiquiatría y Salud Mental, Hospital Clínico San Carlos, Madrid, España. laura_reyes_molon@yahoo.es
L
L
Reyes Molón
R M
RM
Yáñez Sáez
M I
MI
López-Ibor Alcocer
D000293:Adolescent; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis / Q000628:therapy