but. Although very few people have heard of it It's the No. 1 inherited neuromuscular disease in
America. Charcot-Marie-Tooth disease (CMT) is an inherited neurological disease characterized
by gradual degeneration of nerves, which starts in the hands and feet and results in numbness,
muscle weakness and loss of function.
Charcot-Marie-Tooth (CMT) disease typically occurs in 1 person per 2,500. The disease is not
fatal , however , the effects that it causes on the body is very detrimental , especially to the
individual 's self-confidence. The disease involves damage to the covering myelin sheath around
nerve fibers. In some, the disease causes destruction of the myelin sheath. In others, the central
(axon) portion of the nerve cell wears away (Charcot, 1992). CMT is one of 40 diseases covered by the Muscular Dystrophy Association (MDA) but unlike
muscular dystrophy, in which the defect is in the muscles, CMT is a disorder in which the defect
is in the nerves that control the muscles. CMT disease is a form of mutation of the genetic
makeup of the individual . It particularly affects the genes which are responsible for the
production of genes and which are involved in the proper functioning of the peripheral nerve
axon. Peripheral nerves carry movement and sensation signals between brain and spinal cord
and the rest of the body (Kronig, 1984).
Symptoms usually start around the teen years. For reasons unknown, the severity in
symptoms can also vary greatly, even among members of the same family. Generally, CMT is
not a totally debilitating condition but it can make life exhausting and more challenging. The
symptoms of CMT are progressive. The first sign of CMT is generally a high arched foot or
problems with walking. Other symptoms of the disorder may include foot
References: Briggs, J. R., and Freehafer, A. A. 1967 : Fusion of the Charcot spine. 53: 83-93, Brown, C Culling, J., and Gibberd, F. B Charcot 's disease of the spine. Proc. Roy. Soc. Med., 67: 1026-1027, 1974. Daher, Y. H.; Lonstein, J. E.; Winter, R. B.; and Bradford, D. S.: Spinal deformities in patients with Charcot-Marie-Tooth disease. A review of 12 patients. Clin. Orthop., 202: 219-222, 1986. Kronig, G. Spondylolisthese bei einem Tabiker. VII (fase Supplement): 165, 1884. Lee, C. K., and Langrana, N. A. Lumbosacral spinal fusion. A biomechanical study. Spine, 9: 574-581, 1984.