Tourettism as clinical presentation of Huntington's disease with onset in childhood

Infantile Huntington's disease (HD) shows a wide clinical heterogeneity. He re we describe the case of a child affected by HD who showed unusual neurol ogical features consistent with tourettism. The absence of family history a nd persisting normal magnetic resonance imaging (MRI) results long after th e onset of symptoms delayed the diagnosis of the disease. An MRI exam perfo rmed 26 months; after disease onset disclosed bilateral atrophy in the puta men, suggesting HD. The diagnosis was confirmed by genetic analysis. The pr esent report underlines the need to consider HD in childhood cases of unusu al and even unfamiliar progressive movement disorders.