What are the AAN/AANEM guidelines for monitoring and treatment of limb-girdle or distal muscular dystrophy?

Updated: Aug 17, 2020
  • Author: Twee T Do, MD; Chief Editor: Jeffrey D Thomson, MD  more...
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Monitor patients for the development of spinal deformities to prevent resultant complications and preserve function (level B)

Refer patients with musculoskeletal spine deformities to an orthopedic spine surgeon for monitoring and surgical intervention if it is deemed necessary in order to maintain normal posture, assist mobility, maintain cardiopulmonary function, and optimize quality of life (level B)

Refer patients to a clinic that has access to multiple specialties (eg, physical therapy, occupational therapy, respiratory therapy, speech and swallowing therapy, cardiology, pulmonology, orthopedics, and genetics) designed specifically to care for patients with MD and other neuromuscular disorders in order to provide efficient and effective long-term care (level B)

Clinicians should recommend that patients have periodic assessments by a physical and occupational therapist for symptomatic and preventive screening (level B)

While respecting and protecting patient autonomy, clinicians should proactively anticipate and facilitate patient and family decision-making as the disease progresses, including decisions regarding loss of mobility, need for assistance with activities of daily living, medical complications, and end-of-life care (level B)

Prescribe physical and occupational therapy, as well as bracing and assistive devices that are adapted specifically to the patient's deficiencies and contractures, in order to preserve mobility and function and prevent contractures (level B)

Advise patients that aerobic exercise combined with a supervised submaximal strength training program is probably safe (level C)

Advise patients that gentle, low-impact aerobic exercise (swimming, stationary bicycling) improves cardiovascular performance, increases muscle efficiency, and lessens fatigue (level C)

Counsel patients to hydrate adequately, not to exercise to exhaustion, and to avoid supramaximal, high-intensity exercise (level C)

Educate patients who are participating in an exercise program about the warning signs of overwork weakness and myoglobinuria, which include feeling weaker rather than stronger within 30 minutes after exercise, excessive muscle soreness 24-48 hours after exercise, severe muscle cramping, heaviness in the extremities, and prolonged shortness of breath (level B)

Clinicians should not offer patients gene therapy, myoblast transplantation, neutralizing antibody to myostatin, or growth hormone outside of a research study designed to determine the efficacy and safety of the treatment (level R)

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