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

T. Kyriakides, C. Angelini, J. Schaefer, S. Sacconi, G. Siciliano, J. J. Vilchez, D. Hilton-Jones

Research output: Contribution to journalReview articlepeer-review

108 Citations (Scopus)


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 ≥3× 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.

Original languageEnglish
Pages (from-to)767-773
Number of pages7
JournalEuropean Journal of Neurology
Issue number6
Publication statusPublished - 2010


  • Asymptomatic
  • HyperC-Kemia
  • Investigation
  • Pauci-symptomatic


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