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At a glance

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.

Synonym

None.

History

Individuals with this condition were first found in the Moosehead Lake region of Maine and the Canadian province of Quebec among an isolated population of lumberjacks of French Canadian descent. It was first described in 1878 by George Miller Beard (May 8, 1839-January 23, 1883), an American neurologist, who popularized the term neurasthenia around 1869.

Incidence

It is known to affect men more often than women. While the disorder was common in the lumber camps of the Maine region during the 19th and early 20th centuries, it was also observed in specific isolated populations in Louisiana where it is called Rajun Cajuns Disease, in Malaysia where it is called Latah Disease, and in Siberia, India, Somalia, Yemen, and the Philippines where it is called Myriachit Disease.

Genetic inheritance

Rare. There are questionable genetic components. Perhaps a psychological disease described as “operant conditioning.”

Clinical aspects

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 and sporadic. 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 (ie, the “parrot” response).

Anesthetic considerations

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

Other conditions to be considered

  • Gilles de la Tourette Syndrome: Characterized by repetitive compulsive involuntary stereotyped movements or vocalizations termed “tics.” The first symptoms usually are seen during childhood and defined as rapid eye blinking or facial grimaces. Symptoms may also include involuntary movements of the extremities, shoulders, face, and voluntary muscles. Onset usually occurs before the age of 16. It has been suggested that Jumping Frenchman of Maine Syndrome may be a variant of the Gilles de la Tourette Syndrome.

  • KOK Disease (Hyperexplexia Syndrome): Rare, hereditary, neurological disorder that affects neonates. It can also be noticed in the fetal period. It may also affect children and adults. It is characterized by excessive startle reaction associated with sudden unexpected noise, movement, or touch. Symptoms include muscle stiffness or hypertonia. Hyperreflexia and unstable gait may also occur. Because the disorder is frequently misdiagnosed as a form of epilepsy, the process of getting an accurate diagnosis may be ...

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