Comprehensive treatment of CMT

There is no specific drug treatment for CMT, which is primarily a supportive symptomatic treatment that often requires close multidisciplinary cooperation. Research in the treatment of CMT has focused on the development of new treatment strategies, and animal and clinical trials have been conducted with a number of drugs. 1. rehabilitation, orthopedic and supportive treatment Different rehabilitation therapies have been used to treat CMT. passive muscle stretching exercises to maintain normal posture and balance play an important role in preventing tendon contractures and maintaining normal gait; patients with CMT have reduced peak oxygen consumption and reduced aerobic capacity. mild to moderate exercise exercises are safe and effective for patients with CMT and can improve aerobic capacity, and can Mild to moderate exercise is safe and effective for CMT patients, improving aerobic capacity and significantly improving walking ability and lower extremity muscle strength, but high-intensity training should be avoided as much as possible; shoe supports, orthopedic shoes and assistive devices are helpful in preventing the progression of deformity and improving walking ability. Shoe braces are often used to maintain the correct position of the foot, thus avoiding pressure sores and calluses. Ankle-foot orthoses are often used to overcome foot drop and reduce falls to facilitate walking, but are often not tolerated because they are very uncomfortable. Custom-fitted ankle-foot orthoses tend to be more comfortable, reduce the pain of bowed feet, and have better compliance, and brace orthoses are used in cases of severe upper extremity involvement. Respiratory failure due to diaphragmatic weakness or vocal cord paralysis is rarer and has been reported in some CMT1A, CMT2C, and other types of CMT. Ventilation can be improved by assisted ventilation, laser arytenoid chondroidectomy, etc. 2.Surgical treatment Different surgical methods have been used to treat skeletal deformities, especially foot surgery has been performed.CMT foot deformity is a progressive process, with flexible high arched pronated foot deformity in childhood and adolescent patients, which gradually progresses to a fixed deformity with age. Alternative surgical options include soft tissue surgery, osteotomy, and joint fusion, either alone or in combination. Soft tissue surgery includes plantar fasciotomy (to reduce the arch deformity), various types of tendon transfers (peroneus longus-peroneus shortus, anterior tibial septum-posterior tibial septum, etc.), and Achilles tendon lengthening; various types of osteotomies are used when the horseshoe high arch pronation deformity is severe or fixed, mostly on the heel, metatarsal (especially the first), metatarsal tarsal, and tarsal bones; and tri-articular fusion of the talar, talar navicular, and heel dice joints. Joint fusion is used to treat the most severe foot deformities. The long-term results of foot surgery need to be studied prospectively and retrospectively to determine the optimal timing and surgical approach for patients. Upper extremity tendon transfers are also used to help restore thumb position and wrist extension. 15-25% of CMT patients have scoliosis, and in cases where the deformity is very severe patients also require surgical orthopedic treatment. 3, symptomatic drug treatment Pain is a common symptom in CMT patients, mainly related to osteoarthrosis, skeletal deformities and postural abnormalities, partly related to muscle fatigue, and partly to neuralgia. Treatment includes physical therapy, shoe braces to improve foot posture, foot surgery if necessary, and pharmacotherapy with anti-inflammatory analgesics and neuralgic painkillers; fatigue is often seen in CMT patients and may be related to different factors such as decreased muscle strength, impaired cardiopulmonary function and obstructive sleep apnea syndrome. The stimulant modafinil has been effectively used to treat fatigue symptoms in four patients with CMT1A, but because of its nonspecific central excitatory effects and associated side effects, discretion is still needed when administering the drug; CMT patients should avoid drugs that cause peripheral neurotoxicity, especially chemotherapeutic agents such as cisplatin, oxaliplatin, vincristine, and paclitaxel derivatives. Cases of acute neuropathy similar to that of Green-Barre syndrome have been reported in patients with undiagnosed CMT due to the use of vincristine.