Huntington's Chorea in Childhood

Abstract

Huntington's chorea is generally considered to be a disease of the adult. In a recent analysis of 762 patients, Wendt¹calculated the mean age of onset of clinical manifestations at 44 years of age and reported that in 64% of the patients the onset was between 35 and 55 years. However, cases with onset before the age of ten years have been mentioned repeatedly in the extensive medical literature on Huntington's chorea. The description of these patients is usually brief and often incomplete with little or no follow-up. In only a few reports was the clinical diagnosis confirmed by pathological examination. Clinical recognition of the disease in children may offer considerable difficulty because typical choreatic movements are often absent, the presenting symptomatology being one of hypokinesia, muscular rigidity, epilepsy, and mental retardation. The purpose of this report is to describe the clinical, pathological, and genetical data of four children