Paolo Vinci M.D.: Specialist in Physical Medicine and Rehabilitation - Professor of the School for Physiotherapists- University of Rome-Italy; Medical Manager Physiatric and Orthopedic Specialized Hospital of Ariccia (Rome)-Italy

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Improvement of muscle strength, resistance and balance Management of deformities Promotion of mobility: Contractures Orthoses Prevention of Falls Improvement Of Hand Function Management Of Respiratory Failure Solutions to Psychological Problems Solutions to Other Problems

1. Improvement of muscle strength, resistance and balance ·0 If the muscle to be strengthened is only able to contract with no resistance of any kind, a strengthening exercise program is either ineffective or may cause increased weakness. ·0 If your muscle is able to contract with weight applied,you should see a physiotherapist to find out the maximum weight the muscle is able to tolerate. There was a study done of exercise in Neuromuscular Diseases, by Dr.W. Fowler Jr of the Rehabilitation and Research Training Centre in Neuromuscular Diseases at UC Davis. The muscle can then be exercised three days a week, starting with three sets of four repetitions; this can be increased over a twelve week period to eight repetitions per set. A modest increase in strength may result.  ·0 Strengthening exercises which use heavy weights should always be avoided: increased weakness and muscle cramps may occur. ·0 It is very important to avoid immobilization, if possible, because it causes disuse muscle atrophy as well as loss of the motor patterns you have learned, which compensate for the weakness in some muscle groups: it is advisable to be physically active for 2 or 3 hours every day. ·0 Exercises designed to improve coordination and balance can help, especially after a period of bed rest. (e.g. after illness or a fracture requiring rest in bed). ·0 It is impossible to reduce the tiring of your muscles: the only therapy is... rest.   ·0 Leading the most normal life possible is the best active therapy 2. Management of deformities and contractures Bone and joint deformities are caused by muscle contractures. Deformities are caused by an imbalance of strength in the muscles that act across joints. In CMT, the dorsiflexor muscles [tibialis anterior, peroneus longus and brevis],(which pull your foot up) are affected more than the plantarflexor muscles [tibialis posterior and gastrocnemius],(which push your foot down). As CMT progresses, dorsiflexion of the foot becomes impossible and you get foot drop. Sometimes the heel will not reach the floor, [pes equinnus] while you are standing. (Your achilles tendon has a contracture). Usually, this is associated with rotation of your foot, [pes equinnus-cavus-varum-supinatum] causing a reduction in size of the area of your foot which contacts the floor. This causes pain along the outside border of your foot. The same mechanism may occur in the wrists causing a claw hand deformity.  Contractures can help be prevented by: ·0 Stretching calf muscles: the best stretching is obtained by standing and walking, with flat shoes and foot orthoses. ·0 Wear night braces: to keep your feet in the proper position while you are asleep. ·0 Avoid the weight of blankets on your feet: a light duvet or a bar for the blankets are recommended, especially during adolescence.  When stretching is no longer effective and your heel will not reach the ground when you are standing, surgical Achilles lengthening (or other surgical procedures) are necessary. If a contracture forms in one foot only, standing position may be altered and scoliosis may develop. Contractures may occur in your hands, leading to claw hand deformity: a daily self-made stretching exercise of your fingers and wrists is useful to prevent them becoming fixed in one position. Muscle contractures may cause deformities that can only be corrected with bone surgery, especially if they occur while the bones are still growing. Pages:1    2

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