Abstract:
Dentatorubral pallidoluysian atrophy (DRPLA) is a rare
type of an autosomal dominant progressive late onset
cerebellar ataxia. It is caused by a defect in a gene [CAG
repeat in atrophin-1 (ATN1) on chromosome 12] and results
in damage to the cerebellum, and to its connections within
the central nervous system. It is also known as Haw River
Syndrome and Naito-Oyanagi disease. This disorder seems
to be very rare except in Japan. Patients with DRPLA can
exhibit a variety of psychiatric symptoms in addition to
extrapyramidal and cerebellar symptoms. We report a case