Introduction

Abstract

Since the late 1880s hereditary chorea is named Huntington’s disease after George Huntington (1850–1916) (Fig. 1.1) who provided the classical description of adult onset of hereditary chorea in his essay “On chorea” from 1872. However, this publication was not the first clinical description of adult-onset hereditary chorea. It was preceded by earlier and fairly complete clinical descriptions (e.g., that of Waters, Lund, or Lyon) which already mentioned many of the key clinical features of the disease including its hereditary nature, insidious onset in adulthood, and progressive disease course (Heathfield 1973; Huntington 1872; Lanska 2000, 2010; Lund 1860; Lyon 1863; Waters 1842). In his essay from 1872, Huntington incorporated the medical records of the patients treated previously by his father and grandfather and noted the hereditary transmission of chorea, its gradual onset during adulthood and progressive course, the tendency of affected patients to insanity and suicide, as well as the resistance of the disease to treatments. In its main part this historical essay dealt with rheumatic or Sydenham’s chorea, but its last part was devoted to a hereditary chorea with mental changes commencing in midlife, which is transmitted from generation to generation and follows a remorselessly progressive course (Heathfield 1973; Huntington 1872; Lanska 2000, 2010).