$Unique_ID{BRK02083}
$Pretitle{}
$Title{Charcot-Marie-Tooth Syndrome or Progressive Neural Muscular Atrophy}
$Subject{Charcot-Marie-Tooth Syndrome progressive neural muscular atrophy
myelin nerve nerves nervous system muscles brace orthopedic support devices
Charcot Marie Tooth muscle dystrophy muscles}
$Volume{}
$Log{
Anatomy of Nerves*0008101.scf}

Copyright (c) 1993  Tribune Media Services, Inc.


Charcot-Marie-Tooth Syndrome or Progressive Neural Muscular Atrophy


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QUESTION:  I have an elderly friend who wears braces on his feet because he
has no feeling in them.  The doctor's have told him he has a rare disease
called "Marie Shark Tooth Syndrome".  He said that was all he was told.  We
would appreciate any information you may have on this.

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ANSWER:  Medical terms are confusing enough, but when the names of the
discoverers are used to identify the condition, it becomes even more
bewildering.  The actual name of the syndrome derived from the names of three
physicians who first described the disease in 1886 is Charcot (nothing to do
with sharks), Marie (a last name, not the name of a woman), Tooth (nothing to
do with teeth), thus Charcot-Marie-Tooth Syndrome, but perhaps better
identified as "progressive neural muscular atrophy".  It is a disease that
attacks the myelin of nerves, a substance that acts much the same as
insulation on electrical wire.  When this substances disappears as the result
of the disease, the nerve can no longer conduct impulses which, in turn,
effects the muscles.  Though arms and hands can be affected it is the legs and
feet which are most often involved.  Gradually muscles of the leg lose
strength and mass.  A brace is used to help keep the foot in place when the
muscles can no longer do so.  It is the most common inherited nerve disease,
and since it is transmitted by a single dominant trait a family history of the
disease can frequently be discovered.  It is usually first seen in puberty or
early adult life.  The condition progresses slowly, is seldom totally
incapacitating, and often comes to a halt.  The only treatment is orthopedic
support devices which further explains the need for the brace.

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The material contained here is "FOR INFORMATION ONLY" and should not replace
the counsel and advice of your personal physician.  Promptly consulting your
doctor is the best path to a quick and successful resolution of any medical
problem.