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.
Rare. Questionable genetic
component. Perhaps a psychological disease described as “operant
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).
No references. Premedication prior to
anesthesia may be of some value. Consider meticulous padding and
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
Howard R, Ford R: From the Jumping Frenchmen of Maine to post-traumatic
stress disorder: The startle response in neuropsychiatry. Psychol Med
Saint-Hilaire MH, Saint-Hilaire JM. Jumping Frenchmen of Maine. Mov Disord
Saint-Hilaire MH, Saint-Hilaire JM, Granger L: Jumping Frenchmen of Maine.