Neurodegenerative disorders

Abstract

Huntington disease (HD) is an autosomal dominant neurodegenerative disorder clinically characterized by the presence of choreiform movements, psychiatric sequelae, and dementia. While the majority (>90%) of HD patients become clinically symptomatic in adulthood, 5% to 10% of patients present with the juvenile-onset form of the disease, which is almost invariably associated with inheritance of the mutant allele from a symptomatic father. Unlike the adult-onset form of the disease, juvenile HD is generally characterized by the presence of progressive rigidity, seizures, ataxia, and dystonia. © 2007 Springer Science+Business Media, LLC.