Disease Information for Jumping Frenchmenof Maine (unco)

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Clinical Manifestations
Signs & Symptoms
Myoclonus/seizure triggered by stimuli/touch/sound
Startle Myoclonus
Startle response/trigger
Syncope/Collapse with startle reaction
Demographics & Risk Factors
Travel, Geographic & Climate Related Factors
Residence/Travel/Canada
Residence/travel/New England
Ethnic or Racial Factors
French population/ethnic stock
French-canadian ethnic stock
Associated Diseases & Rule outs
Rule Outs
Epilepsy
Associated Disease & Complications
Jumping Frenchmen of Maine
Disease Mechanism & Classification
Pathophysiology
Pathophysiology/Extreme startle reaction
Process
PROCESS/Hereditofamilial (category)
Synonyms
Synonym
Jumping Frenchmenof Maine, Synonym/Jumping Frenchmen, Synonym/Latah (Malaya), Synonym/Myriachit (Siberia)
Definition

Jumping Frenchmen of Maine; Jumping Frenchmen; Latah (Observed in Malaysia); Myriachit (Observed in Siberia); "Jumping Frenchmen" is a disorder characterized by an unusually extreme startle reaction; The startle reaction is a natural response to an unexpected noise or sight; This disorder was first identified during the late nineteenth century in Maine and the Canadian province of Quebec; Lumberjacks of French Canadian descent were originally associated with this phenomenon but it has since been observed in other societies in many parts of the world as well; "Jumping Frenchmen" is suspected to be a genetic disorder and/or an extreme conditioned response to a particular situation possibly influenced by cultural factors; Symptoms tend to improve with age; likely recessive hereditary disorder (NORD 2005)-----------------------------------------

Jumping Frenchmen of Maine is a rare disorder originally described by G M Beard in 1878; It results in an exaggerated "startle" reflex, and was first noted among related French-Canadian lumberjacks in the Moosehead Lake area of Maine; It is not clear if the disorder is neurological or psychological; The "Jumping Frenchmen" seemed to react abnormally to sudden stimuli; Beard recorded, for instance, individuals who would obey any command given suddenly, even if it meant striking a loved one, and repeat back unfamiliar or foreign phrases uncontrollably; Beard also noticed that the condition was often shared within a family, suggesting that it was inherited; The interest Jumping Frenchmen of Maine sparked by Beard"s publication about the disease inspired Georges Gilles de la Tourette to investigate what later became known as Tourette"s syndrome; Further studies of the condition in the 1980s, however, cast doubt on whether the "Jumping Frenchmen" phenomenon was in fact a physical condition like Tourette"s; Documentation of direct observation of "Jumping Frenchmen" has been scarce, and while videotape evidence was recorded by several researchers that showed the condition to be real, Saint-Hilaire concluded from studying eight affected people that it was brought on by conditions at their lumber camps and was psychological, not neurological-----[medical geography website 2005]----------

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NEJM (The New England Journal of Medicine)
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