Physical Medicine and Rehabilitation for Limb-Girdle Muscular Dystrophy Treatment & Management

Updated: Apr 19, 2016
  • Author: Vinod Sahgal, MD; Chief Editor: Stephen Kishner, MD, MHA  more...
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Treatment

Rehabilitation Program

Physical Therapy

The natural history of limb-girdle muscular dystrophy is one of gradual progression over years, with life expectancy beyond the fifth and sixth decades of life. The age of onset of the disease varies from childhood to adult life. Considering these significant differences, the goals of management must vary. With childhood onset, especially during the growth period, the goal of therapy is aggressive prevention of contractures at the hip and shoulder girdle, via stretching. [15, 49]

Exercise program

Very few studies detail the effectiveness of an exercise regimen in limb-girdle syndrome. Given the slowly progressive nature of the disease, the prudent approach to exercise therapy is to prescribe active-assistive and resistive movements and preserve and maintain muscle strength in the pelvic and shoulder girdle musculature. This therapy can prevent the rapid development of orthopedic deformities of hyperlordosis, pelvic forward rotation, and flexion/abduction contracture. In spite of the active exercise regimen, some patients need taping, orthotic devices, and surgical interventions, because of increasing pelvic deformity, hip flexion contractures, and equinus deformity. The exercise regimen should be monitored clinically by watching for the development of leg cramps while biochemical measurements of myoglobinuria, creatinuria, and/or CK (ie, for elevation) are performed. (See Surgical Intervention.)

The active-assistive and resistive exercise regimen also provides hemodynamic stability and avoids hemodynamic decompensation from immobility and cardiomyopathy.

A study by Jensen et al found that in patients severely affected with Becker or limb-girdle muscular dystrophy, combined strength and aerobic antigravity training led to improved closed-kinetic-chain leg muscle strength, although without improvement in isometric knee extension strength or absolute rate of force development. The study included eight patients, who underwent training three times per week for 10 weeks. [50]

A Turkish study examined the effects of breathing exercises and threshold muscle training on maximal inspiratory and expiratory pressures in patients with either limb-girdle or Becker muscular dystrophy. [51] The authors concluded that exercise training in ambulatory patients with muscular dystrophy can increase the patients' respiratory muscle strength and suggested that such therapy be included in their treatment.

Wheelchair prescription

If the patient becomes nonambulatory, wheelchair mobility is essential. The wheelchair should complement the patient's lifestyle, providing comfort, safety, and functionality. Because of the functional weakness and contractures in the upper and lower extremities of patients with limb-girdle dystrophy, special attention should be given to the frame, seat, backrest, front rigging, rear wheels, and casters. An accessible home and work environment and personal or public transportation with safe restraint systems for the wheelchair are also important. (See Further Outpatient Care.)

Occupational Therapy

Similar treatment programs, especially focusing on the shoulder, should be instituted in the upper extremities in patients with limb-girdle muscular dystrophy. The maintenance of active range of motion and strength results in independence in performance of activities of daily living, such as dressing, oral/facial hygiene, homemaking, and preparation for work.

Recreational Therapy

Because of the lifelong impact of limb-girdle muscular dystrophy, adaptations that allow avocational pursuits are essential, and the role of recreational or child-life therapists is important.

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Surgical Intervention

Patients with limb-girdle muscular dystrophy (LGMD) who develop an equinus foot deformity can benefit from tendon-lengthening surgery and/or knee-ankle-foot orthoses or ankle-foot orthoses to maintain mobility.

A surgical approach has been attempted to correct the flexion contractures and scoliosis only in persons with Duchenne dystrophy. Results have been conflicting, because after surgery, patients often are unable to maintain their ambulatory status. These surgical approaches have been tried sparingly in cases of LGMD, and no control studies have been conducted; however, in a few isolated reports, good results in the maintenance of ambulation have been reported after surgery.

In exceptional cases of shoulder-girdle involvement, the patient may benefit from scapulopexy (attaching the inner border of the scapula to the fourth rib using either Mersilene tape or fascia lata). The goal of these interventions is to maintain ambulation and shoulder-girdle function for as long as possible.

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