Infantile Huntington's disease.

A unique case of Huntington's disease is reported because of the extremely early onset and death, and the atypical mode of presentation including severe behavioural problems and a negative family history. Although rare, Huntington's disease must be considered along with the established degenerative disorders of white and gray matter peculiar to the pediatric population when one examines an infant or child with progressive motor deterioration, rigidity, mental retardation and behavioural abnormalities. Computed tomography is a reliable and non-invasive method of establishing the diagnosis during life.