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How Common Is Charcot Marie Tooth Or Is It A Rare Disease?
Charcot Marie Tooth ( CMT ) disease is the most coarse inherit muscular disorder . It is an inherited disorder that feign both motor and sensorial heart without any grounds of an implicit in metabolic disorder . It is also known as transmissible motor and sensory neuropathy ( HMSNs ) and they are dissimilar from transmissible sensory neuropathies or hereditary motor neuropathies . The name of the disease is mint after the medico Jean - Martin Charcot and Pierre Marie of France and Howard Henry Tooth of England in 1886 . It can be further subdivide into CMT 1 , CMT 2 , CMT 3 , CMT X and CMT 4.(1 )
CMT is quite a common disease move about 1 person per 2500 universe and about 125,000 person in the United States . The relative incidence of Charcot Marie Tooth 1 is about 15 somebody per 100,000 populations out of which CMT 1A dominates , with an incidence of 10.5 person per 100,000 populations . Charcot Marie Tooth 2 relative incidence is 7 cases per 100,000 population ; whereas CMT X accounts for about 10 - 20 % of all Charcot Marie Tooth cases . In Spain it accounts for 28.2 case per 100,000 population , in Italy , it report for 17.5 cases per 100,000 populations , in Japan , 10.8 cases per 100,000 population are reported . In Norwegians , it is account to affect about 1 in 1214 individuals.(1 )
Cause Of Charcot Marie Tooth Disease
A nerve is responsible for communicating information to a distant situation by the transmitting of electrical signals via axone of a nerve cell . An axon is insulated by a myelin case ( made up of Schwann cell ) , which protect the nervus and prevents the loss of electric signals . If the axon and medulla case are not integral or there is a disruption in any then the heart conduction will be disturb with kerfuffle inactivation of butt muscles and communicating of sensory information from the muscleman to the head . Charcot Marie Tooth is make when there is a mutation in gene that are responsible for for the yield of structural or usable proteins of the axon or the myelin cocktail dress . There are dissimilar mutations in different forms of Charcot Marie Tooth , but all of these lead to unnatural function of the peripheral nerves . This consequently lead to motor nerve degeneration causing weakness of the muscularity and ultimately wasting away of the involved extremity ( hands , implements of war , foot , and legs ) along with sensory boldness disruption result in lessen sensations toward hot , inhuman , and pain.(2 )
The mutant in the genes are generally inherited , either in an autosomal rife pattern or autosomal recessive pattern . Charcot Marie Tooth disorder may also be inherited in an X link up radiation pattern . CMT disease may also lead in a sporadic form , in which the mutation is not extend down by the family , but come in the individual on its own.(2 )
Symptoms Of Charcot Marie Tooth Disease
Charcot Marie Tooth affect both motor and receptive nerves , so the role related to both the types of nerves is affected . The disease unremarkably occurs in the first two decade of life . sluggish progressive muscle weakness of the extremity is notice , especially of the lower extremity ; Charcot Marie Tooth 1A patients may be seen with proximal muscle atrophy and helplessness . The symptom also let in difficulty walking , frequent sprains and frequent tripping due to muscle weakness and with severe muscle helplessness foot fall and steppage is remark . Foot deformities such as pe cavus ( mellow mischievous foot ) and hammertoes are usually noted due to which calluses , ulcers , cellulitis , and lymphangitis may be note . Spinal deformity , such as thoracic scoliosis may also be seen in some patients.(1 )
The weakness of the deal may leave to poor control of digit , poor handwriting , gracelessness , and difficultness with zipping and buttoning and other manipulations colligate with the hands . The sensations are mostly lack in Charcot Marie Tooth patient role , so they do not complain of sensational symptom , such as numbness , but sensations of vibration and proprioception are markedly reduced . sinew cramping , neuropathic painful sensation or musculoskeletal bother may be present . normally , autonomic symptoms are not present , but some patients may complain of them.(1 )
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