(haw) an autosomal dominant neurodegenerative disorder characterized by ataxia, chorea, seizures, and dementia, with onset usually between ages 15 to 30 years, and death within 15 to 25 years. It is a triplet repeat disorder caused by expansion of the CAG triplet repeat in the DPRLA gene encoding the atrophin-1 protein, as occurs in dentatorubral-pallidoluysian atrophy , although there are some differences in clinical expression and pathology, including lack of myoclonic seizures, and extensive demyelinization of the subcortical white matter, basal ganglia calcifications, and neuroaxonal dystrophy.
