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Huntingtons Chorea.
This very debilitating disease associated with North America seems to have stemmed from families who left Bures in 1630

Symptoms
A rare disease of the central nervous system characterized by progressive dementia with grimacing, gesticulation, ataxic movements, finger twitching, speech disorders and other bizarre involuntary movements.
Name
It was named after George Huntington, an American Physician who first identified the disease.


The following extracts were taken from Internet sites dealing with this disease.

Extract No1
In a lecture which he gave to the New York Neurological Society in 1909, George Huntington said:

"Over 50 years ago, in riding with my father on his rounds I saw my first case of "that disorder", which was the way the natives always referred to the dreaded disease. I recall it as vividly as though it had occurred but yesterday. It made a most enduring impression upon my boyish mind, an impression which was the very first impulse to my choosing chorea as my virgin contribution to medical lore. Driving with my father through a wooded road leading from East Hampton to Amagansett, we suddenly came upon two women both bowing, twisting, grimacing. I stared in wonderment, almost in fear. What could it mean?

In 1692, residents of Salem, Massachusetts, stared at the strange “dancing” movements and violent outbursts of temper of some of their neighbors. Horrified, they concluded that anyone with such bizarre behavior must have been flirting with the devil. Two centuries later, New York physician George Huntington determined that some of his patients with strange twitching behaviors were members of families who had been treated by his father and grandfather. Huntington traced the condition to a family of immigrants from Bures in Suffolk, England, who came to Boston in 1630. He noted that if either parent had the disease, one or more offspring suffered from it. In 1872, Huntington fully described the disease that became known as Huntington’s chorea, from the Greek word for “dance”.

Huntington’s chorea, also called Huntington’s disease (HD), is an inherited degenerative brain disorder affecting about 30,000 people in North America. In addition, about 150,000 people are at risk. Because symptoms do not develop until adult life, parents may pass the disease to their offspring without knowing it. Abnormal movements are accompanied by progressive mental deterioration, personality changes, and loss of speech; death usually comes in 10–15 years. Psychological symptoms may begin more than 10 years before complete onset. The dominant gene, affects men and women of all ethnic groups.

Extract No2
George Huntington's paper, however, gave rise to a great interest in the origins of the disease now bearing his name. Its clear hereditary nature, and the established fact that the condition is rarely if ever sporadic led quickly to searches for the original Huntington disease person or community. The most famous, interesting and quite possibly misleading of these efforts was led by Vessie, who in 1932 suggested that the disorder may have originated in Bures, a small Suffolk village in England. Choreics from this locale were sent from England, probably with the aid of paid witch hunters, to the Massachusetts Colony where their disease and social discord arising from it are adequately recorded in a continuous variety of historic records. Fanning out through New England and later Connecticut, females in family lines became victims of the New England Witch Hunts,

Extract No3
Jelliffe and Tilney took up the task of tracing the ancestry of the families concerned, a study concluded some years later by Vessie (1932), who found that they stemmed from two brothers and their families who had left Bures (in Suffolk), England, and then sailed to Boston Bay in 1630. During the intervening three centuries about 1000 descendants of the original settlers were known to have come down with the disease.

Extract No4 ( Dr Thomas Wood of Bures, author of True Thomas)
Ferriers Manor or Ferriers was originally owned by the Cresseners circa 1300, then passed to the Waldegraves (of Smallbridge Hall) and then again by marriage to the Pelhams.
In 1638 Herbet Pelham took his five motherless children and a number of servants and retainers together with shepherds,blacksmiths and ploughmen from the neighbouring villages of Earls Colne, White Colne and Colne Engaine to the New World.

Amongst those who travelled from Bures were two married couples who were almost certainly related ( on the husbands side) to a third couple, two of Pelhams servants.
Dr Vessie in a Journal dated 1932 traced the descendants of these two families and showed that in the fifth generation appeared and "unequivocal evidence of Huntingdons Chorea", a degenerative disease which eventuality leads to a deep state of restless dementia.
(Journal of Nervous and Mental Diseases 1932 p76)



This information is based on the information gained on the internet and are not the factual views of the page author
updated 12.10.09