Short Transverse Myelitis With Neuromyelitis Optica Spectrum Disorders

Objectives:To evaluate the demographic,clinical characteristics and radiological characteristics of short transverse myelitis(STM)in patients with neuromyelitis optica spectrum disorders(NMOSD),and to improve the understanding of rare cases of STM with NMOSD.Methods:All the statistics of patients with NMOSD were retrospectively collected based on the 2015 International Consensus Diagnostic Criteria for NMOSD.The patients were divided into STM group and longitudinally extensive transverse myelitis(LETM)group according to the length of spinal cord lesions at the initial myelitis manifestation of NMOSD.Results: Twenty-two patients who were AQP4-IgG seropositive with an initial STM represented 44.9%(22/49)of initial myelitis episodes among patients with NMOSD,while there are twenty-seven(27/49,55.1%)patients who were act as longitudinally extensive transverse myelitis.The age of onset of the two groups was statistically different(P=0.046<0.05);But no significant differences were found between NMOSD patients with initial STM and LETM patients with respect to sex,ethnicity(P>0.05).Compared with NMOSD patients with LETM,STM patients exhibited minor motor and bowel/bladderdisabilities.Area postrema syndrome such as stubborn hiccups or nausea and vomiting were rare in both STM and LETM groups.The STM patients showed lowered EDSS at attack.Coexisting autoantibodies including antinuclear antibodies(ANA),anti-Ro-52 antibody,anti-SS-A antibody were present in38.1%(8/22)of STM patients,while there were 50%(13/27)in LETM group.Increased BBB permeability was observed in both groups,and there was a statistically significant difference in albumin index between the two groups.Albumin index was positively correlated to the number of segments of spinal cord involved(r = 0.364,P= 0.01).But there was no significant correlated between albumin index and EDSS score or anti-AQP4-IgG titer.However,there was no significant difference of serum anti-AQP4-IgG titer,CSF white blood cell,CSF protein or serum Albumin between the two groups.Conclusion: Short transverse myelitis is not uncommon in NMOSD.When the NMOSD patients is characterized by short-segment transversal myelitis symptoms,the age of onset is higher than that of patients with LETM,and they showing milder motor and rectal/bladder dysfunction;As to the imaging features,the lesion segment in patients with STM are between 1 and 3,and rarely associated with multiple sclerosis-like brain involvement lesions.