I've told a few friends about a rare conditon I learned about more than a decade ago, and most have been skeptical. The disorder was a negligible component of the neurology class I was taking at the time, but so damned funny (especially given the way the professor described it) that it stuck in my mind, and has occasionally rolled off my tongue for no good reason ever since.
I speak, of course, of the Jumping Frenchmen of Maine syndrome. This condition -- first described by George M. Beard in 1878 after he observed the phenomenon in French-Canadian lumberjacks working near Moosehead Lake in Maine -- is characterized by an exaggerated startle response to stimuli. For example, you might sneak up on an afflicted individual and go "BOO!" and he's apt to not only leap to his feet but also to begin flailing his limbs uncontrollably.
The sharper the stimulus, the more marked the response. People can be made to follow commands they would ordinarily resist because they literally react before they can think. For example, you could tell a man to strike hiswife, and if you did so in an especially loud, sharp, authoritarian voice, chances are the command would be obeyed. They also exhibit echolalia, meaning that they will repeat phrases spoken to them, either in their entirety or, if the phrase is long, a string of words toward the end; and echopraxia, or aping the movements of others.
"Jumping" -- also seen in Malaysia and Siberia --has been observed to run strongly in families, and it has been suggested that the condition results from a mutation that prevents the inhibitory neurotransmiter glycine from acting at its receptor inthe spinal cord, and that this mutation in turn is a result of enthusiastic inbreeding in the remote forests of the Pine Tree State and southern Quebec. However, some researchers, observing that the onset of the disorder coincided with relocation to logging camps, believe that the disorder is not in fact neurologic but the result of operant condition stemming from factors in the camp environment.
Regardless, the conditon qualifies as a form of hyperexplexia, the medical term for any exaggerated startle response. Notably, Beard's work inspired a clinician named Gilles de la Tourette (a Frenchman, but not a jumping one or a Mainer) to investigate a related verbal phenomenon seen in some persons, and the result was the emergence of a disorder bearing Tourette's name that is much better known than the "jumping" phenomenon.
Comments
Fascinating, but the last link leads to a PubMed page that gives the abstract and a broken link to the text. (goes to a 404 error)
any chance of a direct link?
Posted by: Alex | June 19, 2007 8:51 AM
There's also Raynaud's disease, and White Fingers disease,
see http://en.wikipedia.org/wiki/Raynaud%27s_disease
and http://en.wikipedia.org/wiki/Vibration_white_finger
which have ruined the career of many a lumberjack.
Posted by: _Arthur | June 19, 2007 9:34 AM
Tourette! Fuck!
Posted by: dc | June 19, 2007 9:38 AM
I believe you misspelled 'hyperekplexia' - you left off the 'k'. It can also be spelled 'hyperexplexia', but I can find no references to 'hypereplexia'.
Posted by: johnny | June 19, 2007 11:16 AM
You're right, Johnny, I meant PUNCH YOUR GRANDMOTHER! to spell it with the "x" and screwed it up. I hate it when I misspell NUKE IRAQ! medical terms.
Posted by: Kevin Beck | June 19, 2007 2:13 PM
I wonder if this is in any way similar to the "fainting goats" that "freeze" and collapse (not faint) upon being startled.
Fainting goats, Wikipedia
Posted by: Susannah | June 19, 2007 2:49 PM
At the moment (admittedly slightly fortified by a fine French dinner and a bottle of local wine), I'm wondering if I'll ever be able to watch the Monty Python sketch again without some really strange images flirting through (whatever is left of) my mind... albeit the vaguely related Monty Python and the Holy Grail is hugely popular here in France, so perhaps I can claim great confusion than usual?
Posted by: blf | June 19, 2007 8:09 PM