244100 JUMPING FRENCHMAN OF MAINE

TABLE OF CONTENTS

     * Text
     * See Also
     * References
     * Clinical Synopsis
     * Oldno
     * Edit History
       
TEXT

   Beard (1878) first studied this disorder, an exaggerated startle
   reflex. 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,
   abnormal reaction occurred. For example, if one of them 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 command. Some, when addressed
   quickly in a language foreign to them, would echo the phrase (Beard,
   1880). The reports by Beard (1878, 1880, 1886) [a][b][c] stimulated
   George 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).
   Writing on the disorder that Beard described, Stevens (1966) cited a
   personal communication describing 5 affected sibs, offspring of a
   French-Canadian fishing guide in Wedgport, 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 substantiate Beard's description of the last
   century. Direct observations of 'jumpers' have been scarce.
   Saint-Hilaire et al. (1986) studied 8 'jumpers' from the Beauce
   region of Quebec. (One of the authors grew up in that region; I grew
   up in the Moosehead Lake region of Maine.) In the 7 men and 1 woman
   they studied, aged 55 to 77, onset ranged from 12 to 20 years,
   averaging 16 years. 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 century. See Kok
   disease (149400).
   
SEE ALSO

   See also: Karp (1981).
   
REFERENCES

    Andermann, F.; Keene, D. L.; Andermann, E. and Quesney, L. F.:
          Startle disease or hyperexplexia: further delineation of the
          syndrome. Brain 103: 985-997, 1980. 
          
   Beard, G. M.:
          Remarks upon 'jumpers or jumping Frenchmen.' J. Nerv. Ment.
          Dis. 5: 526, 1878. 
          
   Beard, G. M.:
          Experiments with the 'jumpers' of Maine. Popular Science
          Monthly 18: 170-178, 1880. 
          
   Beard, G. M.:
          Experiments with 'jumpers' or 'jumping Frenchmen of Maine.' J.
          Nerv. Ment. Dis. 7: 487-490, 1886. 
          
   Karp, L. E.:
          Genetic drift: in appreciation of Jumping Frenchmen. Am. J.
          Med. Genet. 8: 135-136, 1981. (Editorial)
          
   Saint-Hilaire, M.-H.; Saint-Hilaire, J.-M. and Granger, L.:
          Jumping Frenchmen of Maine. Neurology 36: 1269-1271, 1986. 
          
   Stevens, H. F.:
          Jumping Frenchmen of Maine. Arch. Neurol. 12: 311-314, 1966. 
          
CLINICAL SYNOPSIS

   Neuro:
          
          + Exaggerated startle reflex.
          + Abnormal response to sudden sensory input.
          + Echo words of command.
          + Psychologic operant conditioning.
            
   Inheritance:
          
          + Autosomal recessive.
            
   
   
OLDNO

   24410
   
EDIT HISTORY

   Last: 94/6/3
   mimadm: 94/2/19
   davew: 94/6/3