Evolutionarily speaking, the fight or flight response is a hard-wired physiological reaction that has kept us alive for millennia. It can be argued that without that response we may have never traveled from the great plains of Africa to Europe, Asia, and beyond. The process is the same for every person: some external stimulus triggers a cascade of events. First the amygdala signals the hypothalamus. The hypothalamus then triggers the pituitary gland to release adrenocorticotropic hormone (ACTH) and it also triggers the adrenal glands. The adrenal glands release cortisol and adrenaline, causing a cascade of physiological responses. Blood pressure increases, blood glucose levels rise, and the immune system is suppressed. Pupils dilate and the heart pumps quicker, allowing more blood to travel to the muscles. Bronchia dilate, allowing greater air intake. Sweat glands on the skin contract, causing perspiration. Overall, your body is ready to expend more energy. This cascading physiological event happens rapidly and often as a result of a startle response, or startle reflex.

The startle response is a natural, involuntary reaction to stimuli, such as a flash of light or a loud noise.  Once an alarming stimulus occurs, it triggers the sympathetic nervous system and our initial reaction is fairly uniform. Within milliseconds, our head jerks and neck muscles contract. Eye muscles tighten and we blink. We flinch, our shoulders raise, and our arms stiffen. The muscles in our abdomen tighten and our chest flattens; finally, our knees flex. This reaction happens very quickly in under a second, and once the potential danger has been assessed as something benign, we return to a state of homeostasis. Sometimes, an individual’s reaction may be amusing, and for some of us the act of being startled can be invigorating. Every year, millions of people frequent haunted houses and watch scary movies. The Internet is riddled with practical jokes that intentionally scare viewers. And the occasional scare may actually be good for us. In fact, one study found that watching a scary movie can burn nearly 200 calories. It may also reduce appetite and has been suggested to potentially reduce overall stress levels. However, in rare instances, there are some individuals in which this reflex can be dangerous.

Startle Syndromes

The Jumping Frenchmen of Maine falls into the category of startle syndromes. It has been suggested that many disorders are related to this syndrome, including general anxiety disorders, some forms of Tourette’s, tic disorders, specific startle-induced epilepsy, and others. However, a more common framework to start with would be hyperekplexia, or the “stiff baby syndrome.” Hyperekplexia can be divided into 2 general categories: major or early onset, where there is a definitive genetic link, and minor or late onset, where the root cause is not as clear.

In early onset, babies are born with an exaggerated startle reflex response to auditory or tactile stimuli. The extent of rigidity varies. There have been documented cases where babies become as stiff as a board, to the point that they can be held upright or vertically without a change in posture. They are hypertonic and may present with generalized stiffness, particularly when touched, but the response will subside rather quickly in the absence of stimuli. However, they may be prone to prolonged episodes of rigidity, aspiration, and apnea, which increase the risks of sudden infant death. Cyanosis has been observed in some neonates. In those instances, the Vigevano maneuver, consisting of a forced flexion of the head and legs toward the trunk, has been demonstrated to reverse tonic attacks. Early onset is typically inherited in an autosomal dominant manner. However, recessive and sporadic occurrences are well documented. The most common mutation is in the GLRA1 gene (about 80% of the time), but there are 3 other genes associated with this condition. GLRA1 is associated with malfunctions in the glycine receptors responsible for hyperpolarization and synaptic inhibition in the brain stem and spinal cord. Symptoms typically improve with age but adults have exaggerated startle responses and may walk with a wide-based gate. They may have unusual, automatic defense postures, as described by Suhren while studying an affected Dutch family in 1966.

Adult onset is not as serious and it is still not clear whether or not there is a genetic or any other organic factor. However, the afflicted also suffer from an exaggerated startle response. When startled, their legs stiffen, causing them to fall. There is some debate surrounding the adult onset. Without any structural or genetic cause, some suggest it is a learned behavior and should be classified as such. In many of the late-onset cases, other family members are afflicted with an organic startle disorder. As a side note, hyperekplexia or startle syndromes may have given rise to the saying “scarred stiff,” which originated around 1900.

 Jumping Frenchmen of Maine and Culturally Specific Startle Syndromes

Jumping Frenchmen of Maine may be one of the strangest startle syndromes. It was first described in 1880 by George Beard in the Journal of Nervous and Mental Disease. He observed an isolated group of French Canadian lumberjacks living around the Moosehead Lake district in Maine. About 50 of them displayed very unusual behavior. Their symptoms appeared after puberty and consisted of flailing their arms, jumping, screaming, cursing, and throwing objects. Beard goes on to describe even stranger behavior,

“…The individuals were not able to prevent themselves from starting, striking, dropping, jumping and repeating words or sounds once another person startled them with sudden exclamations or commands. Some, when addressed quickly in a language foreign to them, would echo the phrase, even to the point of quoting from the Odyssey or Iliad. If one person was suddenly asked to strike another, he would do so without hesitation, even when it was his mother and he had an axe in his hand.”

To date, there have been about 70 cases of Jumping Frenchmen of Maine. However, their numbers have decreased to such a point that the phenomenon is virtually nonexistent. Because of the unusual symptoms associated with the Jumping Frenchmen of Maine and the fact that it is isolated to such a small group of people, many consider it to be a culturally specific startle syndrome. Other culturally specific startle syndromes include Latah in Indonesia, Myriachit in Siberia, Yaun in Burma, Bah-Tsche in Thailand, Mali-Mali in the Philippines, the raging Cajuns in Louisiana, Ainu in Japan, Leaping Ague of Scotland, and several others. However, recent literature lacks sufficient references for the majority of these, with the exception of Latah, Myriachit, and the Jumping Frenchmen of Maine. There are similarities across these conditions. In Latah, forced obedience is also observed. Similar to the Jumping Frenchmen, blurting out obscenities or repeating occurs, particularly repeating what startled them (eg, a tiger). However, Latahs sit on the bottom of the socioeconomic ladder and are primarily middle-aged women and/or homosexual transvestites. Another glaring difference is the age of onset. In Latah, onset occurs later in life, whereas in the Jumping Frenchmen of Maine, it is in early childhood/puberty. It has been suggested that Latah behavior is more consistent with a learned and/or reward-based response. In many instances, the peculiar behavior occurs without a startling incident, which has led many to believe it is conditioned and not pathological. However, as with hyperekplexia, there may be two similar but separate conditions: one that has a neuropsychological/organic origin and a second where people merely mimic the behavior of those with the former.

There is a triad of clinical features that can be found in the Jumping Frenchmen of Maine and Latah that includes screaming and/or uncontrolled obscenities, repeating, and obedience to commands. Similarities are also seen in Myriachit, which translates as “doing something stupid and useless because of fear.” Some associate Myriachit with arctic hysteria, as it occurs in areas frequented by harsh winters, with symptoms becoming more acute in times of famine. However, few researchers have observed the behaviors directly, and until the 20th century, Myriachit was more of a curiosity. It was first written about in the late 19th century. William Hammond, a surgeon/neurologist, collected stories from American soldiers stationed in Siberia. Again, there is a striking similarity to the Jumping Frenchmen of Maine. Hammond wrote,

“…To annoy him [the patient, a steward of the boat], some of the passengers imitated pigs grunting, or called out absurd names; others clapped their hands and shouted, jumped, or threw their hats on the deck suddenly, and the poor steward, suddenly startled, would echo them all precisely, and sometimes several consecutively.”

A more disturbing account of Myriachit was written by Shirokogoroff in 1934: “One day he [the subject] was sitting alone, with his small son, in the wigwam. A knife fell down in front of him (evidently the knife had been in the hanging hunting belt). He seized it and thrust it into his son’s body.”

Another unusual situation was observed by Shirokogoroff. A group of solders began to repeat whatever the commanding offer said, obscenities and all. Unlike Latah and the Jumping Frenchmen, spontaneous group infection was never recorded. Because of this, some believe that groups of people who are trained to obey are more susceptible to this strange occurrence. 

As alluded to before, the number of cases of Jumping Frenchmen of Maine has fallen in recent years. It has been suggested that the condition may have begun as a way to break up the boredom of that logging community. There will always be some debate over the origins of culturally specific startle syndromes. It may be part nature and part nurture, and as seen with Shirokogoroff, and under the right circumstances, highly infectious.

Reference

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