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Charcot-Marie-Tooth Disease: An Inherited Neurological Disease, Not a Dental Problem

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Charcot-Marie-Tooth Disease: An Inherited Neurological Disease, Not a Dental Problem
Charcot-Marie-Tooth is not a dental problem. The name may sound funny, yet the disease is anything

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.

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