Huntington’s Disease

Abstract

Abstract Huntington’s disease is the focus of one of the most remarkable stories of discovery in medical history. In 1872, George Huntington, a North American general practitioner, published the first unambiguous description of this unrelenting neurodegenerative disorder in The Medical and Surgical Reporter (Huntington 1872). The title of his article was simply “On Chorea,” and his description of this type of chorea as a specific hereditary disorder became quickly and widely accepted and became known as Huntington’s disease (HD). HD is a chronic, progressive hereditary disease, usually of adult onset, and is typified by a number of motor abnormalities, most obviously the excessive, spontaneous irregular movements called chorea, but also other motor disorders such as rigidity. Accompanying these motor disorders is a range of psychiatric, behavioral and cognitive disturbances and a progressive decline to a severe dementia is almost inevitable if the patient lives long enough.