19. Diagnostic Approach to Pauci- or Asymptomatic hyperCKemia

  1. Nils Erik Gilhus MD, PHD8,9,
  2. Michael P. Barnes MD, FRCP10,11 and
  3. Michael Brainin MD12,13,14
  1. T. Kyriakides1,
  2. C. Angelini2,
  3. J. Schaefer3,
  4. S. Sacconi4,
  5. G. Siciliano5,
  6. J. J. Vilchez6 and
  7. D. Hilton-Jones7

Published Online: 21 SEP 2011

DOI: 10.1002/9781444346268.ch19

European Handbook of Neurological Management, Volume 2, Second Edition

European Handbook of Neurological Management, Volume 2, Second Edition

How to Cite

Kyriakides, T., Angelini, C., Schaefer, J., Sacconi, S., Siciliano, G., Vilchez, J. J. and Hilton-Jones, D. (2011) Diagnostic Approach to Pauci- or Asymptomatic hyperCKemia, in European Handbook of Neurological Management, Volume 2, Second Edition (eds N. E. Gilhus, M. P. Barnes and M. Brainin), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444346268.ch19

Editor Information

  1. 8

    Department of Clinical Medicine, University of Bergen, Norway

  2. 9

    Department of Neurology, Haukeland University Hospital, Bergen, Norway

  3. 10

    University of Newcastle, Newcastle upon Tyne, UK

  4. 11

    Hunters Moor Neurorehabilitation Ltd, Newcastle upon Tyne, UK

  5. 12

    Department of Clinical Medicine and Prevention, Austria

  6. 13

    Center for Clinical Neurosciences, Donau-Universität Krems, Austria

  7. 14

    Department of Neurology, Landesklinikum Donauregion Tulln, Tulln, Austria

Author Information

  1. 1

    The Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus

  2. 2

    University of Padova, Padova, Italy

  3. 3

    Uniklinikum C. G.Carus, University of Dresden, Dresden, Germany

  4. 4

    Nice University Hospital, Nice, France

  5. 5

    Section of Neurology, University of Pisa, Pisa, Italy

  6. 6

    Hospital Universitari La Fe, Valencia, Spain

  7. 7

    John Radcliffe Hospital, Oxford, UK

Publication History

  1. Published Online: 21 SEP 2011
  2. Published Print: 30 SEP 2011

ISBN Information

Print ISBN: 9781405185349

Online ISBN: 9781444346268



  • diagnostic approach to pauci- or asymptomatic hyperCKemia;
  • practical definition of hyperCKemia-upper reference value of CK, above which further investigation being appropriate;
  • sensitivity, likelihood of abnormal results-in somebody with a muscle disease;
  • non-myopathic hyperCKemia-serum CK activity, elevation in patients with skeletal muscle disease;
  • guidelines, diagnostic approach-in patients with paucior asymptomatic hyperCKemia;
  • variables, that increase likelihood-of a diagnostic muscle biopsy;
  • diagnoses in patients-with pauci or asymptomatic hyperCKemia;
  • prognosis in patients-with pauci- or a symptomatic hyperCKemia;
  • risk for malignant hyperthermia (MH) susceptibility-and central core disease;
  • pragmatic approach, advising patients-with otherwise unexplained hyperCKemia


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

Background: Recent epidemiological 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 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 papers identified describe class IV studies.

Recommendations:HyperCKemia needs to be redefined as values beyond 1.5 times the upper limit of normal (which also 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 x 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.