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Charcot Marie Tooth ( CMT ) disease is the commonest neuromuscular disorder that is inherited as it dissemble 1 in 2500 individuals in the United States . It lie in of inherited neuropathies that are unrelated to any metabolic disorderliness . Therefore , they are also known as hereditary motor and receptive neuropathy . The disease was coin after the doc who first distinguish it ; namely , Jean - Martin Charcot , Pierre Marie and Howard Henry Tooth in 1886 . Charcot Marie Tooth consists of a grouping of genetically different diseases having a similar clinical presentation . It is divided into different types that let in Charcot Marie Tooth 1 , CMT 2 , CMT 3 , CMT 4 and CMT X. All are demyelinate neuropathy , except Charcot Marie Tooth 2 , which is an axonal disorder . These are further divided into unlike subtypes.(1 )
Charcot Marie Tooth is mostly see in individuals in their first or second decade of life . It is associated with gradual impuissance of the muscles of the distal extremities along with wasting . This may go to frequent tripping , ankle sprains , walking difficultness , steppage , foot drop , infantry deformities including pes cavus and hammertoe . When the hand is involved , symptoms with difficulty in hired hand manipulations such as zipping , buttoning , penning , poor finger mastery are note . Generally , sensory symptom are not present due to the want of sensations.(1 )
Management Of Charcot Marie Tooth Disease
Till engagement , Charcot Marie Tooth is an incurable disease and can only be make out with various interventions and necessitate multiple subject field including neurology , physical medicine , and rehabilitation , orthopaedics , physical therapy , orthotics , genetics counseling , and psychiatry . Currently , no intervention exists to prevent the loss of myelin cocktail dress , so safe sympathy of genetics and biochemistry of the disease gives hope for next handling considerations . presently , insert andorthoticsare the best potential nonoperative therapy for infantry deformities.(1 )
patient having moderate to severe Charcot Marie Tooth disease can be managed with surgery . The toes that are clawed can be straighten out , feet with high arch can be flattened and ankles that are not straight can be tightened . In the past , the only operating theater that was practiced for Charcot Marie Tooth was the fusion of one or more joints in the foot . Since the advancement in the direction of Charcot Marie Tooth , joint spinal fusion is rarely required ; however , it is important to intervene early that can be started with osteotomies to correct bony misalignment , tendon transfer for foot equilibrium , and ligament reconstructive memory for articulatio talocruralis stabilization ; these can better the markedly meliorate the role and prevent further deformity.(2 )
The clawing of toes is due to weakening of the intrinsic muscles of the foot and tibialis prior brawn of the leg . This lead to hyperextension of the metatarsal - phalangeal stick and flexion of the humble articulation causing pain on the ball of the infantry . This makes walk and wearing shoes hard . former symptoms can be managed with cushioned orthotics , and shoe change ; however , progressed disease required surgical intervention . The soft tissue contractures release causes straightening of metatarsal - phalangeal joints and toes.(2 )
The high arches of the base lead to gait instability , pain on the colloidal suspension of the invertebrate foot and increase predisposition to an ankle sprain . surgical process helps in flattening of the foot that help oneself in even distribution of the stress on the soles of the animal foot along with the repositioning of the heel beneath the weight unit - bearing axis vertebra of the leg . Osteotomy , removal of Proto-Indo European - shaped wedge laterally along with tightening of the lax ankle ligament corrects the high arch that relieves the painfulness and increase endurance and stability of the gait.(2 )
Transfer of tendons , particularly early during the disease phase reduces the malformation advance . Since peroneus brevis tendon tends to weaken , the transfer of peroneus longus assist in maintaining the strength and avoiding deformity of the median midfoot and increasing stableness during stand and walk . The transfer of posterior tibial tendon can increase the forcefulness of ankle and remove the indigence for a drop foot twosome . Even flaccid paralysis of most muscleman groups in the branch benefit from tendon transfer.(2 )
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