Jumping Frenchmen of Maine | |
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Specialty | Medical genetics |
The Jumping Frenchmen of Maine were a group of 19th-century lumberjacks who exhibited a rare disorder of unknown origin. [1] The syndrome entails an exaggerated startle reflex [2] which may be described as an uncontrollable "jump." Individuals with this condition could exhibit sudden movements in all parts of the body. Jumping Frenchmen syndrome shares some symptoms with other startle disorders.
Individuals with this condition were first found in the Moosehead Lake region of Maine, [3] and were first described by George Miller Beard in 1878.
George Miller Beard recorded individuals who would obey any command given suddenly, even if it meant striking a loved one; the Jumping Frenchmen seemed to react abnormally to sudden stimuli. [3] The more common and less intense symptoms consisted of jumping, yelling, and hitting. These individuals exhibited outrageous bursts, and many described themselves as ticklish and shy. Other cases involved echolalia (repeating vocalizations made by another person) and echopraxia (repeating movements made by another person). [4] [5] [6] Beard noted that the men were "suggestible" [7] and that they "could not help repeating the word or sounds that came from the person that ordered them any more than they could help striking, dropping, throwing, jumping, or starting." [7]
The cause of Jumping Frenchmen syndrome is unknown. [8] One theory is that it is a genetic condition. [9] An observation of 50 cases found the disorder to be remotely located and concentrated in the northern regions of Maine. Fourteen of these cases were found in four families. [3] Another set of cases were found in a single family where the father, his two sons, and his two grandchildren exhibited "jumping" behavior. [6]
It may also be a culture-bound syndrome, mass psychogenic illness, 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 being shy may imply that the "jumper" was positively reinforced by the sudden attention as the entertainment for a group. [4]
In 1885, Georges Gilles de la Tourette included Jumping Frenchmen syndrome in the typology of "convulsive tic illness"; [7] studies of the condition in the 1980s cast doubt on whether the phenomenon was in fact a physical condition similar to Tourette syndrome. Documentation of direct observation of "Jumping Frenchmen" has been scarce, and while video evidence was recorded by several researchers that showed the condition to be real, MH and JM Saint-Hilaire concluded from studying eight affected people that it was brought on by conditions at their lumber camps and was psychological, not neurological. [10]
Jumping Frenchmen of Maine syndrome must be distinguished from other conditions involving the startle reflex or tics.
Tourette syndrome is characterized by multiple physical (motor) tics and at least one vocal (phonic) tic. There are many overlaps when compared clinically, but the abnormal "jumping" response is always provoked, unlike the involuntary tics in Tourette syndrome. [9]
Beard had a unique interest in unusual disorders and took the opportunity to observe the epidemic in Maine. [9] He recorded "startle, jumping, and tic-like behaviors" [7] among the French Canadians and lumberjacks who lived near Moosehead Lake in northern Maine. [9] He published his descriptions of the Jumping Frenchmen in 1880, and he believed the condition was hereditary. [7] History of Medicine professor Howard I. Kushner calls Beard's description "the most influential and detailed study" of these behaviors. [7]
According to Kushner, the French physician Jean-Martin Charcot chose his resident, Georges Gilles de la Tourette, to investigate the "relationship between tic disorders and jumping and startle behaviors reported in Malaysia, Siberia, and Maine"; [11] Gilles de la Tourette translated Beard's descriptions and published them one year after Beard's papers. [7] In 1885, Tourette published "Study of a Nervous Affliction" where he included the startle disorders in the typology of what he called "convulsive tic illness", that included what later came to be known as Tourette syndrome. [7] [10] Kushner argues that none of the patients studied by Tourette supported this assertion, [7] and says that "many of his contemporaries refuted Gilles de la Tourette's typology." [12]
Tourette syndrome or Tourette's syndrome is a common neurodevelopmental disorder that begins in childhood or adolescence. It is characterized by multiple movement (motor) tics and at least one vocal (phonic) tic. Common tics are blinking, coughing, throat clearing, sniffing, and facial movements. These are typically preceded by an unwanted urge or sensation in the affected muscles known as a premonitory urge, can sometimes be suppressed temporarily, and characteristically change in location, strength, and frequency. Tourette's is at the more severe end of a spectrum of tic disorders. The tics often go unnoticed by casual observers.
Coprolalia is involuntary swearing or the involuntary utterance of obscene words or socially inappropriate and derogatory remarks. The word comes from the Greek κόπρος, meaning "dung, feces", and λαλιά "speech", from λαλεῖν "to talk".
A tic is a sudden and repetitive motor movement or vocalization that is not rhythmic and involves discrete muscle groups. It is typically brief and may resemble a normal behavioral characteristic or gesture.
Latah is a condition in which abnormal behaviors result from a person experiencing a sudden shock or other external stressor almost exclusively having been observed in persons from Southeast Asia. When induced, the affected person typically engages in such behaviors as screaming, cursing, dance movements, uncontrollable laughter, mimicry and command obedience. Physical symptoms include an increased heart rate and profuse sweating, but no clear physiological causality beyond the apparent relationship between sudden shock and/or severe emotional stress have been identified. Since no research has emerged indicating whether the behavior is caused by a genetic disorder unique to those of Southeast Asian ancestry, a set of psychosomatic symptoms triggered by Southwest Asian cultural anthropological factors, or another cause not yet hypothesized, the cause has remained undetermined.
Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections (PANDAS) is a diagnosis for a subset of children with rapid onset of obsessive-compulsive disorder (OCD) or tic disorders. Symptoms are proposed to be caused by group A streptococcal (GAS), and more specifically, group A beta-hemolytic streptococcal (GABHS) infections. OCD and tic disorders are hypothesized to arise in a subset of children as a result of a post-streptococcal autoimmune process. The proposed link between infection and these disorders is that an autoimmune reaction to infection produces antibodies that interfere with basal ganglia function, causing symptom exacerbations, and this autoimmune response results in a broad range of neuropsychiatric symptoms.
Tic disorders are defined in the Diagnostic and Statistical Manual of Mental Disorders (DSM) based on type and duration of tics. Tic disorders are defined similarly by the World Health Organization.
Georges Albert Édouard Brutus Gilles de la Tourette was a French neurologist and the namesake of Tourette syndrome, a neurodevelopmental disorder characterized by tics. His main contributions in medicine were in the fields of hypnotism and hysteria.
Stereotypic movement disorder (SMD) is a motor disorder with onset in childhood involving restrictive and/or repetitive, nonfunctional motor behavior, that markedly interferes with normal activities or results in bodily injury. To be classified as SMD, the behavior in question must not be due to the direct effects of a substance, autism, or another medical condition. The cause of this disorder is not known.
George Miller Beard was an American neurologist who popularized the term neurasthenia, starting around 1869.
Societal and cultural aspects of Tourette syndrome include legal advocacy and health insurance issues, awareness of notable individuals with Tourette syndrome, and treatment of TS in the media and popular culture.
Tourette syndrome is an inherited neurodevelopmental disorder that begins in childhood or adolescence, characterized by the presence of motor and phonic tics. The management of Tourette syndrome has the goal of managing symptoms to achieve optimum functioning, rather than eliminating symptoms; not all persons with Tourette's require treatment, and there is no cure or universally effective medication. Explanation and reassurance alone are often sufficient treatment; education is an important part of any treatment plan.
Causes and origins of Tourette syndrome have not been fully elucidated. Tourette syndrome is an inherited neurodevelopmental disorder that begins in childhood or adolescence, characterized by the presence of multiple motor tics and at least one phonic tic, which characteristically wax and wane. Tourette's syndrome occurs along a spectrum of tic disorders, which includes transient tics and chronic tics.
The Tourette Association of America (TAA), based in Bayside, New York, United States, is a non-profit voluntary organization and the only US health-related organization serving people with Tourette syndrome. It was founded in 1972 as the Tourette Syndrome Association (TSA), later changing its name.
Arthur K. Shapiro, M.D., was an American psychiatrist and expert on Tourette syndrome. His "contributions to the understanding of Tourette syndrome completely changed the prevailing view of this disorder"; he has been described as "the father of modern tic disorder research" and is "revered by his colleagues as the first dean of modern Tourette syndrome researchers".
Tourettism refers to the presence of Tourette-like symptoms in the absence of Tourette syndrome, as the result of other diseases or conditions, known as "secondary causes".
Sensory phenomena are general feelings, urges or bodily sensations. They are present in many conditions including autism spectrum disorders, epilepsy, neuropathy, obsessive–compulsive disorder, pain conditions, tardive syndromes, and tic disorders.
Habit reversal training (HRT) is a "multicomponent behavioral treatment package originally developed to address a wide variety of repetitive behavior disorders".
Tourette syndrome (TS) is an inherited neurological disorder that begins in childhood or adolescence, characterized by the presence of multiple physical (motor) tics and at least one vocal (phonic) tic.
Basal ganglia disease is a group of physical problems that occur when the group of nuclei in the brain known as the basal ganglia fail to properly suppress unwanted movements or to properly prime upper motor neuron circuits to initiate motor function. Research indicates that increased output of the basal ganglia inhibits thalamocortical projection neurons. Proper activation or deactivation of these neurons is an integral component for proper movement. If something causes too much basal ganglia output, then the ventral anterior (VA) and ventral lateral (VL) thalamocortical projection neurons become too inhibited, and one cannot initiate voluntary movement. These disorders are known as hypokinetic disorders. However, a disorder leading to abnormally low output of the basal ganglia leads to reduced inhibition, and thus excitation, of the thalamocortical projection neurons which synapse onto the cortex. This situation leads to an inability to suppress unwanted movements. These disorders are known as hyperkinetic disorders.
A premonitory urge is a sensory phenomenon associated with Tourette syndrome and other tic disorders. Premonitory urges are "uncomfortable feelings or sensations preceding tics that usually are relieved by [a particular] movement".
Kushner, HI. A cursing brain?: The histories of Tourette syndrome. Harvard University Press, 2000. ISBN 0-674-00386-1