Hereditary paroxysmal ataxia

Abstract
From early childhood, 8 patients in a kindred had paroxysmal bouts of ataxia, dysarthria and nystagmus. The disorder was inherited as an autosomal dominant. Attacks occurred weekly and lasted 1-6 h; there were slight cerebellar signs between attacks. Although the etiology was not determined, a serendipitous trial of acetazolamide completely abolished attacks, and all patients have remained free of attacks for as long as 5 yr.