Muscular Dystrophy in a teenager


Q: I had a muscle biopsy and so far only some results came in, consistent with low grade muscular dystrophy. Do I have malignant Hyperthermia?


A:   Muscle can clinically express disease in very few ways: weakness or decreased movement, muscle ache, or abnormal variations in power as a result of physical activity.The main clinical hallmark of neuromuscular disease (muscular dystrophy) is weakness. A muscular dystrophy is a potentially hereditary disease characterized by progressive degeneration of muscle. Many such diseases exist. The classification comprises Duchenne, Becker, and various other types. Some patients with Congenital Myopathy , especially central core disease (CCD) (less frequently with multicore disease) are inclined to develop malignant hyperthermia. However, since the precise diagnosis may not be known, precautions should be taken in all patients with a presumed diagnosis of congenital myopathy. General anesthesia usually triggers a full-blown episode, but excessive heat, neuroleptic drugs, alcohol, or infections may trigger milder episodes. If surgery is required, these patients (and their relatives) should avoid inhaled anesthetics (except nitrous oxide) and succinylcholine. Signs and symptoms of malignant hyperthermia include the following: muscle rigidity, tachycardia, fever, Hyperventilation, high potassiun, high calcium. Death may result from pulmonary edema, coagulopathy, ventricular fibrillation, cerebral edema, or renal failure. So as you can see the Malignant Hyperthermia might be a complication in certain patients with congenital muscular dystrophy.

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