Neurológia pre prax 1/2022

Hereditary chorea with a tendency to insanity and adult onset: 150 years since George Huntington and his disease

Huntington’s disease is a rare autosomal dominant incurable neurodegenerative disease, manifested by dementia, behavioral symptoms, chorea and other motor features. The disease bears his name after George Huntington (1850–1916), representing the third generation physician of Huntington´s family, working in East Hampton on the east part of Long Island, New York. Here, since his childhood, he could see several patients with a hereditary disease, described by him in a paper entitled „On chorea“, published in The Medical and Surgical Reporter in 1872. In this article, he characterized chorea from the clinical point of view, and also the three main features of Huntington’s disease: heredity, tendency to insanity and occurrence in adulthood. Although it has been known that the disease has been described by other authors before Huntington, this paper has brought him fame. In honor of the 150th anniversary of this story, in this review we have focused on George Huntington and historical circumstances that happened in the time around this important milestone in the history of neurology.

Keywords: George Huntington, chorea, On chorea, Huntington´s disease, 150th anniversary