| Jumping Frenchmen of Maine | |
|---|---|
| Classification and external resources | |
| OMIM | 244100 |
Jumping Frenchmen of Maine is a rare psychological or, possibly, a neurological disorder first observed by George Miller Beard in 1878. It entails an exaggerated "startle" reflex [1] which may be described as an uncontrollable "jump" but can also exhibit sudden movements in all parts of the body. Though distinct and unique, this condition also shares similar symptoms with numerous disorders pertaining to startle. Patients with this disorder were first found in the northern regions of Maine; hence, the unusual name for this medical condition.[2]
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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.[2] There have been cases with echolalia, the act of repeating back unfamiliar or foreign phrases uncontrollably and also echopraxia, the act of imitating movements from surrounding people. The more common and less intense symptoms consist of jumping, yelling, and hitting. Although these patients may exhibit such outrageous bursts, many describe themselves as normally ticklish and shy.[3][4][5]
There are possible suggestions that this is a genetic condition.[6] Through Dr. Miller's observations of 50 cases, he found the disorder to be remotely located and concentrated in the northern regions of Maine. Fourteen of his cases were found in four families.[2] Another set of cases were found in a single family where the father, his two sons, as well as his two grandchildren exhibited "jumping" behavior,[5] strongly supporting the idea that it is a hereditary disorder.[7] Others think this is possibly a cultural personality or a formed habit. These French "jumpers" lived in a very remote region and most were lumberjacks. This type of small community would allow for a majority to adapt to this sort of reaction. Also, instances of many originally being shy may imply that the "jumper" was positively reinforced by the sudden attention as the entertainment for a group.[3]
A startle reflex can occur in the body through a combination of actions. A reflex from hearing a sudden loud noise will happen in the primary acoustic startle reflex pathway consisting of three main central synapses, or signals that travel through the brain.
First, there is a synapse from the auditory nerve fibers in the ear to the cochlear root neurons (CRN). These are the first acoustic neurons of the central nervous system. Studies have shown a direct correlation to the amount of decrease of the startle to the number of CRNs that were killed. Second, there is a synapse from the CRN axons to the cells in the nucleus reticularis pontis caudalis (PnC) of the brain. These are neurons that are located in the pons of the brainstem. A study done to disrupt this portion of the pathway by the injection of PnC inhibitory chemicals has shown a dramatic decrease in the amount of startle by about 80 to 90 percent. Third, a synapse occurs from the PnC axons to the motor neurons in the facial motor nucleus or the spinal cord that will directly or indirectly control the movement of muscles. The activation of the facial motor nucleus causes a jerk of the head while an activation in the spinal cord causes the whole body to startle.[8]
There are many various reflexes that can occur simultaneously during a startle response. The fastest reflex recorded happens within the masseter muscle or jaw muscle. The reflex was measured by electromyography which records the electrical activitiy during movement of the muscles. This also showed the latency response or the delay between the stimulus and the response. Recorded was found to be about 14 milliseconds. The blink of the eye which is the reflex of the orbicularis oculi muscle was found to have a latency of about 20 to 40 milliseconds. The Jumping Frenchmen of Maine may exhibit the "jumping" reflex from a combination of muscles to move the head, neck, shoulders, arms, and legs. Out of these, the head is quickest in a movement latency in a range from 60 to 120 milliseconds. The neck then moves almost simultaneously with a latency of 75 to 121 milliseconds. Next, the shoulder jerks at 100 to 121 milliseconds along with the arms at 125 to 195 milliseconds. Lastly the legs responds with a latency of 145 to 395 milliseconds. This type of cascading response correlates to how the synapses travel from the brain and down the spinal cord to activate each motor neuron.[9]
Dr. Miller recorded conditions for the Jumping Frenchmen of Maine found among the French Canadians and lumberjacks who lived near Moosehead Lake in northern Maine.[6]
Dr. George Miller Beard was an American neurologist born on May 8, 1839 to minister Reverend Spencer F. Beard and his wife Lucy A. Leonard in Montville, Connecticut. After finishing his undergraduate degree from Yale, he continued his studies in medicine at the College of Physicians and Surgeons of New York. He had a unique interest in unusual disorders and immediately jumped at the opportunity to observe the endemic in Maine.[6]
Listed below are anecdotes.
A lumberjack was working in his own horse's stall. His friend secretly waited in the stall right next to where his friend was. Then suddenly, he poked and neighed loudly like a horse. At this, the friend jumped away as though his own horse had kicked him.[10]
A twenty-seven-year-old man was sitting in a chair holding a knife to cut his tobacco. Suddenly, he was slapped on the shoulder and was told to throw the knife. Immediately, he threw it straight into a beam in the opposite side of the room. The slap and the command were repeated a moment later while he was filling his pipe. Once again, the pipe flew out of his hand on to the grass. Later, he was startled while told to "strike" and he did so immediately on the cheek.[11]
A sixteen year old boy was standing in the room when he was suddenly startled and told to jump. He jumped at least half a foot from the floor while yelling the command to jump.[11]
"Goosey Gus" was a factory worker in Maryland in the early 1900s. Many of his fellow coworkers learned to make fun of him by poking him at the ribs whenever they walked by. They were able to make him do anything by merely just poking him. Though he was a very quiet and shy guy, people were able to cause him to use extremely foul language to the boss without any hesitation.[11]
There was a transport driver among the farm laborers in South Africa also from the early 1900s. A small but sudden touch caused him to immediately lose control of his movements. He copied any sounds whether words or musical notes, he copied them without any hesitation or fear of the consequences.[11]
Dr. Michael Davis of Emory University continues a great amount of research in this field. Though he is not specifically working with Jumping Frenchmen of Maine disorder, he hopes that his work will bring more understanding to the bizarre movements of a startle response. There are still many questions that need to be answered.[8]
Tourette Syndrome is a genetic neurodevelopmental disorder. It is characterized by multiple physical (motor) tics, or sudden movements, and at least one vocal (phonic) tic. There are many overlaps when compared clinically. One difference, however, is that unlike Tourette Syndrome, the abnormal "jumping" response is always provoked.[6]
Latah from Malaysia is a disorder where one's startle response is similar to a state of trance with repetitive speech or movements.
Miryachit is a disorder found in Siberia that also displays an action similar to "jumping."
Neurasthenia is a disorder with a startle response during periods of great fatigue.
Hyperekplexia is an extremely rare autosomal dominant neurological disease. It results in the mutation of the glycine receptor that inhibit signals to the spinal cord and brain stem. The symptoms start in infancy with hypertonia, an abnormal muscle tension that decreases flexibility, and an exaggerated startle in all ages of life.[6]
The interest sparked by Beard's publication about the disorder 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.[12]