Abstract
In 1980 Rowland first used the term IDIOPATHIC HYPERCKEMIA to describe persons with unexplained persistent elevation of creatine kinase activity with no abnormalities on neurological/clinical investigation, electrophysiological studies, and muscle biopsy. Causes of hyperCKemia are numerous including endocrinological dysfunctions, adverse effects of drugs, existence of makro-CK as well as the disposition for malignant hyperthermia, an inflammatory or (yet) asymptomatic degenerative myopathy. Physiological conditions as age, gender, and race have influence on CK levels. Several retrospective studies in patients with hyperCKemia could identify neuromuscular diseases in 18-28% (in one study more than 50%) on histopathological analysis, including some cases of inflammatory myopathy. By first-hand experience it is reasonable to perform a muscle biopsy in asymptomatic or mildly symptomatic individuals with hyperCKemia if there is a history for rhabdomyolisis, if EMG studies show myopathic changes and/or creatine kinase activity is markedly elevated. To ensure an optimal diagnostic procedure the biopsy should be carried out in a specialized center.
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