Becker's muscular dystrophy: Rhabdomy

Advanced, Clinical - Neuromuscular Diseases and Disorders

Muscular dystrophy is a group of hereditary diseases characterized by painless degeneration and atrophy of skeletal muscles. There is symmetric skeletal muscle weakness/wasting without denervation (sensation and reflexes are intact).  X-linked recessive types (Duchenne and Becker muscular dystrophies) are the most prevalent types of muscular dystrophy.  Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are caused by a recessive mutation on the X-chromosome that prevents normal formation of dystrophin, a muscle stabilizing protein.  Complete loss of dystrophin (as in DMD) or a partially functional dystrophin protein (as in BMD) disrupts sarcolemma integrity leading to myofibril atrophy, necrosis, and fibrosis.  BMD progresses at a slower rate than DMD with most BMD patients presenting with weakness in adolescence and most DMD patients presenting with weakness and motor delay in childhood (often delayed walking).

In a large literature review of anesthetic complications involving patients with muscular dystrophy, Gurnaney et al. (2009) identified three patients with BMD who received a volatile anesthetic without succinylcholine and developed anesthesia-related cardiac arrest (one intraoperatively, two in the recovery room).  All patients received dantrolene to treat presumed MH; however, a diagnosis of MH was thought to be unlikely in the absence of a hypermetabolic state preceding the rhabdomyolysis and hyperkalemic event (that led to cardiac arrest).  The pathophysiology of inhaled anesthetic-induced rhabdomyolysis in patients with DMD and BMD is unknown; it has been hypothesized that inhaled anesthetics exacerbate a breakdown of the muscle membranes in these patients.  Of note, the notion of volatile anesthetic-induced rhabdomyolysis in patients with muscular dystrophy has been questioned (2) despite recommendations from the American College of Chest Physicians and the American Academy of Pediatrics to avoid volatile anesthetics in these patients (3,4).

Sources

    Hines, R.L., and Marschall, K.E. Chapter 21. Skin and Musculoskeletal Diseases. Stoelting’s Anesthesia and Co-Existing Disease, 6e. 444-446.

    Keys to the Cart: July 23, 2018: a 5-min. ABA Keyword review

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Author
Kevin Greer, MD