eMedicine Specialties > Neurology > Neuromuscular Diseases

Limb-Girdle Muscular Dystrophy: Treatment & Medication

Author: Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Contributor Information and Disclosures

Updated: Nov 28, 2007

Treatment

Medical Care

  • No specific treatment is available for any of the LGMD syndromes, though aggressive supportive care is essential to preserve muscle function, maximize functional ability, and prolong life expectancy.
    • Primary concerns include the prevention and correction of skeletal abnormalities, such as scoliosis and contractures, and the preservation of ambulation.
    • Aggressive use of passive stretching, bracing, and orthopedic procedures allow the patient to remain independent for as long as possible.
  • Cardiopulmonary complications are another concern.
    • Early intervention to treat cardiac and respiratory insufficiency (possibly with intermittent positive pressure ventilation bilevel positive airway pressure [BiPAP] and/or continuous positive airway pressure [CPAP] at times), can help improve function and prolong the patient's life expectancy.
    • Cardiac arrhythmias can be a major cause of morbidity and mortality (sudden cardiac death) in LGMD1B and 1E. Placement of a pacemaker can be a life-saving procedure.
    • A cardiologist or pulmonologist evaluates the patient at least yearly if he or she has symptoms of cardiac or pulmonary disease.
  • As for other hereditary myopathies, a team approach, including a neurologist, pulmonologist, cardiologist, orthopedic surgeon, physiatrist, physical therapist, orthotist, and counselors, ensures the best therapeutic program.

Surgical Care

Orthopedic surgery may be needed to help correct or prevent contractures and scoliosis.

Consultations

  • Orthopedic surgeon
  • Pulmonologist
  • Cardiologist
  • Physiatrist
  • Physical therapist
  • Orthotist

Activity

In general patients with LGMD lead a sedentary lifestyle due to their weakness. The effect of endurance training has been only rarely studied.

  • A study of endurance training on patients with LGMD2I and mild weakness was carried out. The patients cycled for 30 minute training sessions progressing up to a maximum of 5 sessions per week over 12 weeks at 65% of their maximum oxygen uptake. Training significantly improved work capacity, paralleled by self-reported improvements. Creatine kinase levels did not increase significantly, and muscle morphology was unaffected. The authors concluded that moderate-intensity endurance training is a safe method to increase exercise performance and daily function in patients with LGMD2I.
  • However, this was a small study, performed in only one form of LGMD, has not been replicated and lasted only 12 weeks. The long-term repercussions of endurance training in LGMD are not known and caution should be used in recommending endurance training for patients with LGMD.

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
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Further Reading

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Keywords

limb-girdle muscular dystrophy, sarcoglycanopathy, alpha-dystroglycanopathy, LGMD, LGMD1, LGMD2, LGMD2C, LGMD2D, LGMD2E, LGMD2F, myofibrillar myopathy, desmin-storage myopathy,  LGMD2A, calpainopathy, LGMD2B, dysferlinopathy, telethoninopathy, TRIM32 -related dystrophy, LGMD2J, titinopathy, myotilinopathy, laminopathy, caveolinopathy, desminopathy, alpha-β-crystallinopathy, myotilinopathy, filamin C myopathy, selenoprotein N myopathy, laminopathy, LGMD2J, titin protein

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Contributor Information and Disclosures

Author

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Medical Editor

Raj D Sheth, MD, Professor, Departments of Neurology and Pediatrics, Director of Comprehensive Epilepsy Program, Department of Neurology, University of Wisconsin at Madison
Raj D Sheth, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

Agapito S Lorenzo, MD, Laboratory Director, Associate Professor, Departments of Neurology, Creighton University and University of Nebraska Medical Center
Agapito S Lorenzo, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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