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Neurologic disease characterized by exaggerated, and often violent, startle reflexes that are produced by the slightest stimulus. It is usually associated with echolalia and echopraxia.

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Rare. Questionable genetic component. Perhaps a psychological disease described as “operant conditioning.”

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Seemingly a disease isolated to French Canadians, particularly lumberjacks from the Moosehead region of Maine and the Beauce region of Quebec. The condition is often familial. Age of onset is usually between 12 and 20 years. Affected persons have an exaggerated startle reflex produced by the slightest stimulus; if given a short, sudden, quick command, they respond with the appropriate action, often echoing the words of the command (echolalia and echopraxia), even if it was expressed in a language foreign to the patient (i.e., the “parrot” response).

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No references. Premedication prior to anesthesia may be of some value. Consider meticulous padding and immobilization.

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Gilles de la Tourette Syndrome: Repetitive compulsive involuntary stereotyped movements or vocalizations termed tics; for some authors, the jumping Frenchman of Maine syndrome is included in Gilles de la Tourette syndrome.

Howard R, Ford R: From the Jumping Frenchmen of Maine to post-traumatic stress disorder: The startle response in neuropsychiatry. Psychol Med 22:695, 1992.  [PubMed: 1410093]
Saint-Hilaire MH, Saint-Hilaire JM. Jumping Frenchmen of Maine. Mov Disord 16:530, 2001.  [PubMed: 11391751]
Saint-Hilaire MH, Saint-Hilaire JM, Granger L: Jumping Frenchmen of Maine. Neurology 36:1269, 1986.  [PubMed: 3528919]

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