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One may not be able to associate the phrase "Jumping Frenchmen of Maine" with a medical disorder going by its name alone. This rare condition was first observed by George Beard in French Canadian lumberjacks living in the American state of Maine and the Canadian territory of Quebec in the late 19th century.

The condition was characterised by an extreme startle response which included jumping uncontrollably in the air, copying the behaviour of those around them or sudden movement in different body parts when people were surprised or startled. This condition is extremely rare, although it has been reported in various places around the world since.

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Startle responses are reactions when someone is suddenly stimulated. The responses could be vocal or audible, physical or visual or otherwise. There are several startle responses and some can be signs of different neurological or psychiatric conditions. Here are a few examples:

  • Jumping Frenchmen of Maine
  • Hyperekplexia
  • Reflex epilepsy
  • Paroxysmal kinesigenic dyskinesias
  • Episodic ataxia
  • Cataplexy
  • Latah
  • Myriachit

Read more: Narcolepsy

According to a write-up in the Handbook of Clinical Neurology in 2011, hyperkinetic movement disorders like startle syndrome are "characterized by an abnormal startle response that is relayed in the brainstem and likely involves a pathological exaggeration of the physiological startle reflex". Some of the above mentioned disorders are characteristic of the different ethnic groups around the world, where the specific conditions have been reported. Some of them also have similar clinical presentations. 

Read more: Meige syndrome

The Jumping Frenchmen of Maine is one such condition where there is an exaggerated startle response to a sight or a sound. Read on to know more about this extremely rare condition.

  1. Jumping Frenchmen of Maine symptoms
  2. Jumping Frenchmen of Maine causes
  3. Diagnosis of Jumping Frenchmen of Maine
  4. Risk factors of Jumping Frenchmen of Maine
  5. Doctors for Jumping Frenchmen of Maine

Jumping Frenchmen of Maine symptoms

A rare condition reported in only a few parts of the world, Jumping Frenchmen of Maine is characterised by different startle responses. The symptoms of this condition usually begin at a young age (10-13 years).

According to the paper titled "The Enigma of the 'Jumping Frenchmen of Maine'", published in the Maine History Journal, the symptoms include a "combination of jumping, screaming, swearing, flailing out and striking bystanders, and throwing objects that happened to be in their hands". But for a brief moment, these exaggerated responses also included "'automatic obedience': subjects briefly did whatever they were told".

Jumping Frenchmen of Maine causes

There are several theories about the cause of Jumping Frenchmen syndrome, but none of them has been able to conclusively tell us why this condition occurs. One such suggestion is that it is a genetic condition. A 1965 study published in Archives of Neurology (now known as JAMA Neurology) retraced George Beard's original observation of 50 cases in Maine, of which 14 of them were found in just four families, leading to the genetic theory.

Dr Beard wrote that the disorder first appeared in childhood, ran in the family but wasn't seen in females, and was a lifelong condition that included a jumping response to sudden noise or being startled. He further observed that these responses were never random or spontaneous, and did not trigger unconsciousness.

Diagnosis of Jumping Frenchmen of Maine

Diagnosing a condition as rare as Jumping Frenchmen of Maine can be difficult, as it is not only characterised by location or territory, but also through various different symptoms that are also characteristic of other conditions. A neurologist may be required to observe the symptoms who must be able to rule out the possibility of other syndromes such as startle reflex, tics, or even Tourette syndrome.

Risk factors of Jumping Frenchmen of Maine

A study published in the journal Neurology India looking into the startle response disorder known as Hyperekplexia, found that it was easy for doctors to misdiagnose such movement disorders, as the symptoms can sometimes be similar to those seen in epilepsy or seizures.

In countries like India where Jumping Frenchmen is even rarer, diagnosis can be extremely difficult and chances of misdiagnosis quite high.

Other sources have suggested that startle responses like in the case of the Jumping Frenchmen syndrome play a critical role while studying neuropsychiatric conditions because they could point towards greater neurological disorders. 

Prepulse inhibition, for instance, occurs when a subthreshold stimulus - a stimulus that doesn't usually produce an excitable reaction - overtakes a startling stimulus in the brain. This prepulse inhibition is usually seen in various conditions such as schizophreniaobsessive-compulsive disorder (OCD) as well as Huntington's disease.

Dr. Virender K Sheorain

Dr. Virender K Sheorain

Neurology
19 Years of Experience

Dr. Vipul Rastogi

Dr. Vipul Rastogi

Neurology
17 Years of Experience

Dr. Sushil Razdan

Dr. Sushil Razdan

Neurology
46 Years of Experience

Dr. Susant Kumar Bhuyan

Dr. Susant Kumar Bhuyan

Neurology
19 Years of Experience

References

  1. Genetic and Rare Diseases Information Center: National Center for Advancing Translational Studies [Internet]. Gaithersburg, MD, USA. Jumping Frenchmen of Maine.
  2. Saint-Hilaire MH et al. Jumping Frenchmen of Maine. Neurology. 1986 Sep; 36(9).
  3. NORD: National Organization for Rare Disorders [Internet]. Danbury, CT, USA. Jumping Frenchmen of Maine.
  4. Stevens H. "Jumping Frenchmen of Maine" Myriachit Arch Neurol. 1965 Mar; 12(3): 311-314.
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