eMedicine Specialties > Physical Medicine and Rehabilitation > Muscular Dystrophy

Limb-Girdle Muscular Dystrophy: Treatment & Medication

Author: Vinod Sahgal, MD, MS, Chairman, Department of Physical Medicine and Rehabilitation Services, The Cleveland Clinic Foundation; Professor, Department of Physical Medicine and Rehabilitation, Ohio State University
Coauthor(s): Steven Reger, PhD, CP, Professor, Department of Industrial and Manufacturing Engineering, Cleveland State University; Director of Rehabilitation Technology, Department of Physical Medicine and Rehabilitation, Cleveland State University
Contributor Information and Disclosures

Updated: Apr 30, 2009

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.

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 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.43 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.

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.

Medication

No medication is used for the specific treatment of limb-girdle muscular dystrophy.

More on Limb-Girdle Muscular Dystrophy

Overview: Limb-Girdle Muscular Dystrophy
Differential Diagnoses & Workup: Limb-Girdle Muscular Dystrophy
Treatment & Medication: Limb-Girdle Muscular Dystrophy
Follow-up: Limb-Girdle Muscular Dystrophy
Multimedia: Limb-Girdle Muscular Dystrophy
References
Further Reading
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Keywords

limb-girdle muscular dystrophy, muscular dystrophy, dystrophy, muscle disease, muscular disease, polymyositis, spinal muscular atrophy, muscular atrophy, muscular dystrophy symptoms, neuromuscular disease, muscle diseases, limb girdle muscular dystrophy, limb girdle dystrophy, muscular dystrophy causes, neuromuscular diseases, Leyden-Mobius muscular dystrophy, pelvofemoral muscular dystrophy, scapulohumeral muscular dystrophy, LGMD, limb-girdle dystrophy, dystrophia muscularis progressiva, sarcoglycanopathy, sarcoglycanopathies

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Contributor Information and Disclosures

Author

Vinod Sahgal, MD, MS, Chairman, Department of Physical Medicine and Rehabilitation Services, The Cleveland Clinic Foundation; Professor, Department of Physical Medicine and Rehabilitation, Ohio State University
Vinod Sahgal, MD, MS is a member of the following medical societies: American Academy of Neurology, American Academy of Physical Medicine and Rehabilitation, American Congress of Rehabilitation Medicine, American Medical Association, and American Spinal Injury Association
Disclosure: Nothing to disclose.

Coauthor(s)

Steven Reger, PhD, CP, Professor, Department of Industrial and Manufacturing Engineering, Cleveland State University; Director of Rehabilitation Technology, Department of Physical Medicine and Rehabilitation, Cleveland State University
Steven Reger, PhD, CP is a member of the following medical societies: Association for Academic Psychiatry and New York Academy of Sciences
Disclosure: Nothing to disclose.

Medical Editor

Elizabeth A Moberg-Wolff, MD, Associate Professor and Pediatric PM&R Fellowship Director, Department of Physical Medicine and Rehabilitation, Medical College of Wisconsin; Program Director, Tone Management and Mobility, Department of Physical Medicine and Rehabilitation, Children's Hospital of Wisconsin
Elizabeth A Moberg-Wolff, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine and American Academy of Physical Medicine and Rehabilitation
Disclosure: Medtronic Neurological Grant/research funds Speaking and teaching

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kat Kolaski, MD, Assistant Professor, Departments of Orthopedic Surgery and Pediatrics, Wake Forest University School of Medicine
Kat Kolaski, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine and American Academy of Physical Medicine and Rehabilitation
Disclosure: Nothing to disclose.

CME Editor

Kelly L Allen, MD, Regional Medical Director, IMX-Medical Management Services
Disclosure: Nothing to disclose.

Chief Editor

Denise I Campagnolo, MD, MS, Director of Multiple Sclerosis Clinical Research and Staff Physiatrist, Barrow Neurology Clinics, St Joseph's Hospital and Medical Center; Investigator for Barrow Neurology Clinics; Director, NARCOMS Project for Consortium of MS Centers
Denise I Campagnolo, MD, MS is a member of the following medical societies: Alpha Omega Alpha, American Association of Neuromuscular and Electrodiagnostic Medicine, American Paraplegia Society, Association of Academic Physiatrists, and Consortium of Multiple Sclerosis Centers
Disclosure: Teva Neuroscience Honoraria Speaking and teaching; Serono-Pfizer Honoraria Speaking and teaching

 
 
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