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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 Huntingtons chorea, from the Greek word for dance.
Huntingtons chorea, also called Huntingtons
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 1015 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
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