Humans have been designed with
balanced reactions and responses. An excess of any reaction or an exaggerated response
may lead to the diagnosis of a disorder.
Humans have a naturally occurring
startle response. Startle response is a rapid response to a sudden or
unexpected stimulus, for example, a sudden loud sound or a flashlight, etc. It
is a quick, automatic, and protective response by the nervous system which
contracts the muscles and moves the body away from the stimulus.
An exaggerated startle response is a disorder. سباق حصان
Normally, the protective reflex movement away from the
stimulus subsides in a couple of seconds when the person realizes that there is
no danger. But with the genetic mutation, the pathways regulating the startle
response are not regulated; therefore, the startle response is exaggerated and bizarre.
This disorder is called ‘Hyperekplexia’ or ‘Jumping Frenchman of Maine
Disorder.’
The otherwise normal stimulus which would lead to a self-subsiding startle response in normal individuals would make the sufferers of this disease jump, twitch, cry out, or even have convulsions. The response is exaggerated and prolonged. Also, the jumper is susceptible to another episode of jumping or exaggerated startle response right after an episode ends, unlike normal people who do not get startled with the same stimulus right after having one reflex.
What’s with the name ‘Jumping Frenchman of Maine Disorder? كيف تربح المال من النت ’
It is not unusual for a disorder
to get its name from the first physician to describe it or the first patient to
suffer. Similarly, this disease has been named after its first sufferers, a
group of French-Canadian lumberjacks who worked in Northern Maine in the 1870s.
The disease was first described by G. bet356 M. Beard, who, while describing the disease,
wrote:
“One of the
jumpers while sitting in his chair with a knife in his hand was told to throw
it, and he threw it quickly so that it struck in a beam opposite; at the same
time, he repeated the order to throw it…. When the commands are uttered in a
quick, loud voice, the jumper repeats the order. When told to strike, he
strikes, when told to throw it, he throws it, whatever he has in his hands….
all of these phenomena were indeed but parts of the general condition known as
jumping. It was not necessary that the sound should come from a human being:
any sudden or unexpected noise, as the explosion of a gun or pistol, the
falling of a window, or the slamming of the door, provided it be unexpected and
loud enough, would cause the jumpers to exhibit some, one or all of these
phenomena….” (Beard, 1880a, pp. 487-490)
There is no specific test to diagnose
Hyperekplexia; it is a clinical diagnosis, nor is there any conclusive
treatment for it. The response dampens with age provided that stimulus is
avoided by not intentionally teasing or startling. Stress and anxiety may also
increase the frequency of episodes. Pharmacologically, benzodiazepines have
been shown to provide some relief.
