Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neuronal degenerative disease characterized by ataxia, choreoathetosis and cognitive dysfunction, including dementia, resulting from polyglutamine (polyQ) expansion in Atrophin1 (ATN1)19, 20, 21. The gene discussed is ATN1; the disease is dentatorubral-pallidoluysian atrophy.