Serum creatine kinase levels in spinobulbar muscular atrophy and amyotrophic lateral sclerosis

Nizar Chahin, Eric J. Sorenson

Research output: Contribution to journalArticlepeer-review

61 Scopus citations


We compared serum creatine kinase (CK) levels between spinobulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS) and reviewed available histochemical studies of frozen sections of muscle biopsies. CK levels and the frequency of patients with elevated CK levels were significantly higher in the SBMA group when compared with the ALS group. CK levels occasionally approached values up to 8 times the upper limit of normal in the SBMA group. In addition to the chronic neurogenic changes in the muscle biopsy, all SBMA patients showed one or more myopathic changes. Increased numbers of markedly hypertrophic fibers were consistently seen in all patients. It is not clear whether the elevated CK level is directly related to the increased number of hypertrophic fibers or to other myopathic features. Based on these findings, we recommend genetic testing for SBMA in cases of male patients with motor neuron disease who present with a significantly elevated serum creatine kinase level, even when other characteristic clinical features of SBMA are absent.

Original languageEnglish (US)
Pages (from-to)126-129
Number of pages4
JournalMuscle and Nerve
Issue number1
StatePublished - Jul 2009
Externally publishedYes


  • Amyotrophic lateral sclerosis
  • Creatine kinase
  • Hypertrophic fibers
  • Prognosis
  • Spinobulbar muscular atrophy

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)


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