Symptoms
A rare disease
of the central nervous system characterised 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 Herbert 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)
Extract No5
Thus it was possible to determine that the Long Island cases were the
offspring of a man named Mulfoot or Mulford, who had been born and brought
up in the Massachusetts-Connecticut area. The Mulfoot ancestry was then
laboriously traced back to three young men and their wives who, in 1630,
had left the village of Bures St Mary on the border between Suffolk and
Essex, to settle in the American colonies. The Lord of the Manor at Bures
was the wealthy, puritanical Herbert Pelham.
Finding himself increasingly dissatisfied with the High Church proclivities
of the government he decided to emigrate. He offered to give passage to
any villagers who wished to join him. In 1630 Pelham and the three families
embarked in the James Winthrop Fleet, landing three months later in Salem,
Mass.
Pelham was a man of probity but his companions were very different. Even
on the voyage they were in constant trouble on account of their reprehensible
behavior. Once established in their new home, they and their descendants
proved to be delinquents. Intermarriage, illegitimacy and incest were
common: the males were constantly in and out of goal. The fate of the
female members was worse. No fewer than seven of them were arraigned,
punished and even burnt at the stake by reason of witchcraft. Indeed,
the notorious Groton witch was a member of this family.
A study of the transcripts of these early trials specifically refers to
the wild, uninhibited grimacings and contortions, thus indicating that
the alleged demoniacal possession was, in reality, the expression of a
neurological disorder.
Who were the original three families from Bures and what was the real
reason for their escaping to America? We have every reason to suspect
that the three males were blood-relations and possibly their wives too.
A study of the Church records at Bures makes it clear that a certain Mary
Haste was the parent of at least two of these men and almost certainly
of all three.
It is now realized that the descendants of Mary Haste were responsible
for the largest community of cases of Huntington's chorea in America.
Did Huntington's chorea exist among
Mary Haste's forebears? It seems likely, though it cannot be proved.
Essex and Suffolk were blackspots of alleged witchcraft in the sixteenth
and seventeenth centuries. A most careful search of the lists of those
accused of sorcery in East Anglia has uncovered two pairs of victims sharing
names which appear in such far-removed areas as East Anglia and New England.
Reference:- MacDonald Critchley (1984). The history of Huntington's chorea.
Psychological Medicine, 14, pp
Extract No6 :-
Elizabeth Knapp
This is another young lady who seems to take the blame for introducing
the disease into the new World
Elizabeth Knapp was born in Massachusetts in 1655. At age 16, she was
a servant in the household of Reverend Samuel Willard of Groton, Massachusetts
when she first exhibited signs of being possessed by the Devil.
The Knapp family originated from Bures St Mary, England
As her symptoms intensified she fell into violent fits, complained
of being strangled, and attempted to throw herself into the fire
Reverend Willard observed that she began to "carry herself in a strange
and unwonted manner," saw apparitions, and experienced violent "fits"
over a period of three months.
Modern medicine has cast an interesting light on part of this story. It
now appears that Elizabeth most likely was afflicted with adult onset
Chorea, also known as Huntington's disease, a rare dominant genetic disorder
that takes its name from the Greek word for dancer. Eight people in every
100,000 in Europe and North America suffer from it.
The disease causes the selective deterioration
of certain movement related structures deep inside the brain. The symptoms
of the disease, which may first appear around puberty, include excessive,
spontaneous, irregular movements of the limb that flow from one part of
the body to the other that worsen over time, sometimes leading to neurological
deterioration including apathy, irritability, memory loss, manic depression,
and schizophrenia.
Ref:- http://www.womenhistoryblog.com/2008/03/elizabeth-knapp.html
Latest Update April
2021
Taken from research notes made by Dr Brown, the Bures GP until he died
in 1991
Perhaps the hopelessly flawed pedigree
and confused genealogy of Dr Vessie's original article are understandable,
although it destroys this theory that Huntingdons Disease can be traced
back "for three hundred years" to "the Bures family
group".
A certain amount of extra confusion in the story was added by the writing
of Dr Macdonald Crichley. Dr Adrian Caro with the help of his wife Sheila
Haines, a medical research genealogist, of the Research Unit at Dereham
Hospital, Norfolk, published in 1975 the result of three years work on
the history of Huntington's Chorea.
He published the article in "Update" July 1975 "to demonstrate
the lack of evidence to support what has been stated in the past",
that is about Mary Hast. Their main conclusion was "that Mary
Hast has no place in the saga of Huntington's Chorea".
Doctor Caro and his wife concluded "our own research suggests...that
there is no evidence for implicating any one person within this large
group of people." Our conclusion is that at the birth of this
legend fantasy took over and disbelief was suspended. Unthinking obedience
sustained the story and fancy embellished it.
Update September 2021
Contributed by Virginia Easley DeMarce, Falls Church, VA, USA
In relation to Huntington's Disease, the pseudonymic "Jeffers"
in articles in the first half of the 20th century was a man named Jeffrey
Ferris, born circa 1610 somewhere in England, but with no known connection
to Bures St. Mary. His first wife, the mother of his children, is unknown.
The pseudonymic "Wilkie" was a William Knopp, who was not related,
as far as genealogical records provide data, to either Nicholas Knapp
or Jeffrey Ferris. The English origin of Nicholas Knapp is unverified;
he was not from Bures St. Mary, but he hasn't been documented as being
from any of the other suggested locations, either.
Editors
Notes
I have spent several hours researching the possible individuals in the
Bures area, that could have carried this disease to the New World
We seem to have:-
Elizabeth Knapp, referred to as possibly the Grand-daughter of
"Wilkie"
Mary Haste - unknown
Wilkie, Nichols and Jeffers - Two or even the three of these youths,
possible offspring of Mary Haste
With such conflicting opinions on the Internet,
I am not sure it`s possible to say for certain which individual or individuals
are responsible for this outbreak
The choice is yours:-
References:-
Knapp
family in Mount Bures
Select Bibliography
Doctor Adrian Caro and Sheila Haines
"History of Huntington's Chorea" in "Update" (July
1975)
Doctor Macdonald Critchley
"Huntington's Chorea and East Anglia" (1934)
"Huntington's Chorea" (1964)
"Black Hole and Other Essays" (1973) No. 5
This information is based on the information
gained on the internet and donated material, they are not the factual
views of the page author
updated 07/04.2013
updated 26/01/2017
updated 13/04/2021
updated 14/09/2021
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