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Eur J Neurol. 2010 Jun 1;17(6):767-73. doi: 10.1111/j.1468-1331.2010.03012.x. Epub 2010 Apr 5.

EFNS guidelines on the diagnostic approach to pauci- or asymptomatic hyperCKemia.

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1
Clinical Neurosciences, The Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus. theodore@cing.ac.cy

Abstract

OBJECTIVE:

To provide evidence-based guidelines to general neurologists for the assessment of patients with pauci- or asymptomatic hyperCKemia.

BACKGROUND:

Recent epidemiologic studies show that up to 20% of 'normal' individuals have an elevated creatine kinase activity in the serum (sCK). The possibility of a subclinical myopathy is often raised, and patients may be unnecessarily denied treatment with statins.

SEARCH STRATEGY:

Electronic databases including Medline, the Cochrane Library and the American Academy of Neurology were searched for existing guidelines. Articles dealing with series of patients investigated for asymptomatic/pauci-symptomatic hyperCKemia and articles dealing with myopathies that can present with asymptomatic hyperCKemia were identified and reviewed.

RESULTS:

The only guidelines found were those approved by the Italian Association of Myology Committee, and the only relevant articles identified describe class IV studies.

RECOMMENDATIONS:

HyperCKemia needs to be redefined as values beyond 1.5 times the upper limit of normal (which itself needs to be appropriately defined). Pauci- or asymptomatic hyperCKemia with no apparent medical explanation may be investigated with a muscle biopsy if one or more of the following are present; the sCK is >or=3x normal, the electromyogram is myopathic or the patient is <25 years of age. In addition, women with sCK<3 times normal may be offered DNA testing because of the possibility of carrying a dystrophin mutation.

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