Physical Medicine and Rehabilitation for Limb-Girdle Muscular Dystrophy Follow-up

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

Further Outpatient Care

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  • Wheelchair prescription

    • The object of a wheelchair prescription is to extend the functional mobility of the patient while secondarily providing exercise and postural stability that may delay loss of strength and prevent deformity. This requires specific attention to all components of the chair to keep it as lightweight, durable, and functional as possible.

    • Early after the onset of limb-girdle muscular dystrophy, the use of a lightweight manual wheelchair can help to extend the patient's range of travel, even while he or she is still ambulatory. The operating environment, abilities of the user, and progression of the disease must be considered, with careful selection of seat width, seat height, and push rim location relative to the shoulder and arm position of the user. Properly adjusted height-of-the-arm supports can also prolong self-implemented pressure relief (push-up) function using glenohumeral depression with the elbows anchored on the armrest.

    • Later, as the disease progresses, particular attention may focus on power mobility, with tilt-in-space function used to provide independence in the face of poor upper extremity control and loss of independence stance. Off-the-shelf modular components or a custom-contoured wheelchair seat and back inserts may be used.

    • The use of head and neck supports also may become necessary.

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Complications

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  • Contractures

  • Scoliosis

  • Pulmonary problems

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Prognosis

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  • In this group of disorders, the mortality ascribed to the disease and/or the complications thereof are negligible. However, the prognosis in limb-girdle muscular dystrophy with regard to mobility, self-care, and the maintenance of the ability to work is dependent on the aggressive, goal-directed management described in various subsections of this article.

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