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The Jumping Frenchmen of Maine: A Rare Clinical Syndrome

Dec 29, 2022

Background

In response to sudden, unexpected stimuli, human beings and animals exhibit a quick and reflexive startle response.1 In certain cases, however, this startle response can be exceptionally severe.1

The Jumping Frenchmen of Maine disorder was first described by Dr. George Beard in a group of French-Canadian woodsmen from northern Maine.1,2,3 It is a very rare clinical syndrome characterized by an extraordinarily exaggerated startle reaction including jumping, yelling, hitting, automatic obedience to sudden commands, echolalia, and echopraxia.3

Today, Jumping Frenchmen of Maine is one among a group of similar culture-specific disorders called “startle-matching syndromes”, which have been observed in various regions across the world.1,4

Causes and pathogenesis

Jumping Frenchmen of Maine is believed to be a neuropsychiatric disorder, but its exact cause and underlying mechanisms are unknown.1,4 The rarity of this condition has precluded epidemiological investigations and case–control studies.1,4 Previously, it was theorized that this syndrome could be genetic, since 14 of the 50 original cases observed by Beard came from four families.5 Moreover, this disorder was not observed in women.5 However, pedigree analysis could not prove this theory.5  Given that both genetic and environmental factors could contribute to this disorder, the two current theories are as follows1:

Signs and symptoms

In cases of Jumping Frenchmen of Maine, symptom onset typically occurs after puberty or during adolescence.1 Individuals with this disorder display exaggerated startle responses when faced with unexpected or sudden stimuli, including noises, commands or gestures, or physical contact.1 The abnormal startle response includes one or more of the following1:

  • Jumping
  • Screaming
  • Flailing of the arms
  • Hitting
  • Throwing of objects
  • Echolalia, i.e., repetition of words or phrases like a broken record
  • Echopraxia, i.e., imitation of movements or gestures
  • Coprolalia, i.e., involuntarily swearing or utterance of taboo words
  • Forced obedience, i.e., automatic response to simple commands

While symptom frequency tends to decrease with age, intensity increases under stress or anxiety.1 According to Beard, the onset of startle episodes often coincided with stressful conditions, like lumberjack work, and symptom severity and frequency both decreased once the affected individuals left the lumberjack camp.1

Diagnosis

The diagnosis of Jumping Frenchmen of Maine is complicated because symptoms can be indicative of multiple disorders.1 Neurologists typically diagnose this condition based on a careful clinical evaluation, detailed patient history, and a variety of specialized tests for ruling out other similar disorders, such as startle reflex, tics, and Tourette’s syndrome.1,5

Treatment

There are no specific treatments for the Jumping Frenchmen of Maine syndrome.1 Reducing the practice of intentionally startling and/or teasing individuals with this condition to initiate a response could help in reducing or eliminating episodes, especially given the link between symptom intensity and stress.1

References

  1. Jumping Frenchmen of Maine. [Online] NIH-GARD. Available at: https://rarediseases.info.nih.gov/diseases/6803/jumping-frenchmen-of-maine
  2. Richard MP. A peculiar condition: A history of the Jumping Frenchmen Syndrome in scientific and popular accounts. Journal of the History of the Neurosciences. 2018;27(4):355–74.
  3. Saint-Hilaire MH, Saint-Hilaire JM, Granger L. Jumping Frenchmen of Maine. Neurology. 1986;36(9):1269.
  4. Bakker MJ, Van Dijk JG, van den Maagdenberg AM, Tijssen MA. Startle syndromes. The Lancet Neurology. 2006;5(6):513–24.
  5. Jumping Frenchmen of Maine. [Online] MyUpchar. Available at: https://www.myupchar.com/en/disease/jumping-frenchmen-of-maine

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