Clinical characteristics of neuromyelitis optica spectrum disorders associated with syringomyelia

Objective To analyze the clinical characteristics of neuromyelitis optica spectrum disorders (NMOSD) associated with syringomyelia (SML). Methods The clinical manifestations, laboratory parameters and imaging findings of 7 patients with NMOSD associated with SML during June 2008 to August 2016 from The Third Affiliated Hospital of Sun Yat-sen University were retrospectively analyzed. Results Six patients were female and 1 was male, with ages ranging from 27-67 years, the course of the disease was 3-12 years, recurrence was 2-7 times, all the patients were cerebrospinal fluid oligoclonal bands (CSF OCB) negative, 5 patients were positive for aquaporin-4-antibody/NMO-IgG. Sensory and motor dysfunction, bowel or bladder dysfunction were the common clinical manifestation, and pain was a significant symptom. Syringomyelia was predominantly located in C4-T10, cavity length 2-6 mm, diameter 0.8-3.6 mm; 5 had lobes involvement and 6 had long segmental spinal cord involvement. Conclusions NMOSD associated with SML had a high frequency of occurrence in female, with the clinical characteristics of limb pain, easy progression, high recurrence rate and disability rate. The anti AQP4 antibody were commonly seen in these patients. Syringomyelia was predominantly located in the lower cervical and upper thoracic spinal cord, the lobar and longitudinally extensive transverse myelitis were frequently found in patients with NMOSD associated with SML. Key words: Neuromyelitis optica spectrum disorders; Syringomyelia; Aquaporin-4-antibody/NMO-IgG; Clinical characteristics