Serum Creatine Kinase in Patients with Neuromyelitis Optica Spectrum Disorder

Abstract

Objectives: There have been reports of elevated serum creatine kinase (CK) and myopathy in patients with Neuromyelitis Optica Spectrum Disorder (NMOSD). Such findings have raised the possibility that myopathies may be a part of the spectrum of NMOSD. The incidence of elevated CK in NMOSD remains unknown. We sought to assess the potential association between hyperCKemia, myopathy, and NMOSD, and the potential role of screening for muscle involvement using serum CK.

Methods: We reviewed records of all aquaporin 4 (AQP4) antibody-seropositive and seronegative NMOSD patients who had CK levels evaluated at two major academic medical centers.

Results: Of 199 total NMOSD patients, CK levels were checked in 43, and elevated, on at least one occasion, in 4. In 1 patient, CK was elevated during an NMO exacerbation. A myopathic process occurring with NMOSD was suggested in 2 of 4 patients in the form of symptomatic complaint of myalgias and associated MRI signal change.

Discussion: Unexplained hyperCKemia was found on one or more occasion in 4 of 43 tested NMOSD patients. Testing NMOSD patients for serum CK may reveal otherwise unsuspected myopathy. More formally powered, prospective assessment of the incidence and utility of CK in NMOSD is needed.

Keywords: Aquaporin 4; Creatine kinase; Myopathy; Neuromyelitis Optica.