What Is Huntington's Disease?

The "Huntington Disease" or "Huntington's Chorea" is a debilitating neurological disorder of genetic origin characterized by progressive neuronal degeneration in the basal ganglia that is typically manifested in chorea, ie, a rhythmical, involuntary movements and uncontrollable. It seems that the term chorea-Greek etymology, means dance or dance-was already used by Paracelsus to denote this type of involuntary movements.

However, the description of this species as a specific entity morbid was crystallized in the nineteenth century in various investigations until the publication in 1872 of work is today considered the classic reference, On chorea, designed by American physician George Huntington (1850-1916) .

Slightly more than one page in the journal Medical and Surgical Reporter, Philadelphia, gave their name to this disease. It seems that in addition to the accuracy, conciseness and clarity of the description of the disease, identified his familial incidence, which has subsequently been attributed to the inheritance of a particular gene.

For Huntington, precisely what distinguished this condition from other Koreas described hereditary nature was as much his as the late onset of symptoms. George Huntington was only 22 years and only had one who had obtained his license to practice general medicine in the College of Physicians and Surgeons in New York when he published his article.

However, it was preceded by a long family tradition of physicians who practice the profession as he would do in his hometown of Easthampton, Long Island, New York, and his brief experience had assimilated the observations of his father and grandfather about this dramatic disease and the families it affected.

This is what seems to add this feature enabled so important to the characterization of chorea that now bears his name, because, he declared modestly and some ingenuity, no data on the disease out of Long Island and thought that perhaps describing not happen to be a medical curiosity.