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Background: Guillain-Barré syndrome (GBS) is an immune-mediated inflammatory polyneuropathy characterized by acute flaccid paralysis. Elevated creatine kinase (CK) levels in GBS have been reported to be transient, and levels vary from mild to severe. Herein, we report a case of GBS with elevated CK mimicking acute myositis.Case Report: A 48-year-old man presented with pure motor flaccid quadriparesis. Power was 2/5 with hypotonia and areflexia in all four limbs. A nerve conduction study revealed reduced compound muscle action potential in all recorded motor nerves. Serum CK was 2,334 U/L. The patient’s symptoms progressed despite intravenous methylprednisolone administration. Cerebrospinal fluid (day 8) revealed albuminocytological dissociation, and electromyography (day 21) revealed spontaneous activity with neurogenic motor unit action potential suggestive of acute motor axonal neuropathy variant of GBS.Conclusion: In a patient with elevated CK and ascending paralysis, differential diagnosis of GBS should be considered and cerebrospinal fluid study and electromyography should aid in confirming the diagnosis.

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