Muscle disorders

This chapter discusses updated management for the following muscular disorders: duchenne muscular dystrophy (DMD), limb-girdle muscular dystrophies (LGMDs), myasthenia gravis, polymyositis, and facioscapulohumeral muscular dystrophy (FSHD). It presents the background, clinical scenario and evidence for each one of these muscular disorders. Oral corticosteroids are widely accepted as first-line immunosuppressive treatment for autoimmune myasthenia gravis, although there is surprisingly little definitive evidence for their efficacy compared with other treatments. As is the case with steroids, no quality-randomized controlled data exist for any other immunosuppressant therapy. FSHD is the third most common dystrophy following Duchenne's and myotonic dystrophy. Extramuscular manifestations include mild high-frequency hearing loss and retinal telangiectasias. Periscapular weakness prevents even a relatively spared deltoid muscle from elevating the arm. Several retrospective reviews have suggested significant benefits, however no prospective controlled study confirms this.