This disorder, an exaggerated startle reflex, was first described by Beard (1878). His communication was made to the American Neurological Association and consis...
This disorder, an exaggerated startle reflex, was first described by Beard (1878). His communication was made to the American Neurological Association and consisted of observations among French Canadian lumbermen from the Moosehead Lake region of Maine. He noted that the condition was often familial. In response to sudden sensory input, an abnormal reaction occurred. For example, if an affected person was abruptly asked to strike another, he would do so without hesitation, even if it was his mother and he had an ax in his hand. If given a short, sudden, quick command, the affected person would respond with the appropriate action, often echoing the words of the command. Some, when addressed quickly in a language foreign to them, would echo the phrase (Beard, 1880).
Writing on the disorder that Beard described, Stevens (1965) cited a personal communication describing 5 affected sibs, offspring of a French Canadian fishing guide in Wedgeport, Nova Scotia.
Andermann et al. (1980) reported colleagues who had videotaped interviews with several 'jumpers' from the Beauce region of Quebec, an area from which many of the lumbermen of the Moosehead Lake region of Maine came. These tapes presumably substantiated Beard's description of the disorder.
Direct observations of 'jumpers' have been scarce. Saint-Hilaire et al. (1986) studied 8 'jumpers' from the Beauce region of Quebec. In the 7 men and 1 woman they studied, aged 55 to 77, onset ranged from 12 to 20 years of age, averaging 16 years of age. In 6 of 7 men, onset coincided with start of work as a lumberjack and the seventh had worsening of preexisting symptoms when he began work as a lumberjack. In 3 there was a positive family history. Saint-Hilaire et al. (1986) concluded that 'jumping' is not a neurologic disease but rather can be explained in psychologic terms as operant conditioning. The cases they studied were related to specific conditions in lumber camps in the 19th and early 20th centuries.
See hyperekplexia (149400).
The reports by Beard (1878, 1880, 1886) stimulated Georges Gilles de la Tourette to study patients making peculiar sounds and movements and led to description of the disorder his mentor Charcot referred to as Gilles de la Tourette syndrome (137580).
Dr. Victor McKusick grew up in the Moosehead Lake region of Maine.
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