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The history of Huntington's chorea

Probably the first reference in medical literature to the condition known as Huntington's chorea or Huntington's disease appeared in Dunglison's Practice of Medicine, which was first published in 1842. The author's information derived from a letter from the Rev. C. O. Waters referring to a group of residents in the south-east corner of New York State who showed a rare hereditary disorder. The syndrome comprised the onset in adulthood of involuntary movements, unclear speech, advancing over the years to a state of dementia. In Dunglison's third edition (1848) reference was also made to similar cases in and around Philadelphia, recorded by Charles Gorman, and known locally as the 'magrums'. John Christian Lund reported the same disorder affecting members of two families in Saetersdalen, in southern Norway, in one instance involving four generations. The vernacular terms were the 'rykka' (or 'jerks') or else the 'arvesygen' (or 'hereditary disease'). Turning back the pages of medical history we find that in 1633 a certain Simon Huntington, a grocer from Norwich, decided to settle in the New England colonies, patriotically naming his new habitat Norwich, Connecticut. In 1777 Simon Huntington's great-great-great grandson Abel was born. After qualifying as a doctor he moved to East Hampton, Long Island. His son, George Lee, was born in 1811 and subsequently joined his father's practice. In 1850 his grandson George was born, qualifying at New York in 1872 and he, too, joined his father and grandfather in Long Island. He chose the topic of Sydenham's chorea for his M.D. thesis. The final paragraph called attention to the occurrence in East Hampton of a number of families who showed the identical syndrome of jerking movements and mental disorder. All three of the doctors were familiar with these cases and, indeed, the manuscript of the thesis contains marginal notes pencilled in by the father and grandfather. Obviously, they were ignorant of what had previously been published on this disorder. Locally, it was referred to with awe as 'that disorder' for it carried with it a stigma, being popularly attributed to a curse which had befallen a remote ancestor who had mocked the suffering of Christ upon the cross. With the later discovery of other affected families elsewhere in the world, and particularly North America, considerable interest was raised in the genealogy of this disorder which by now had rightly received the eponymous designation of Huntington's chorea. 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 behaviour. 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.