[Clinical presentations of neuromyelitis optica spectrum disorders with ultra-longitudinally extensive transverse myelitis].

ABSTRACT

Objective: To analyze the clinical presentations of neuromyelitis optica spectrum disorders (NMOSD) with ultra-longitudinally extensive transverse myelitis (uLETM), in order to improve the diagnostic accuracy of this disorder. Methods: Twenty-two uLETM patients was recruited and retrospectively analyzed for general clinical characteristics, laboratory tests and MRI characteristics, as well as therapeutic. Results: (1)The Male-to-female ratio was 1∶6. The median onset age was 31 years old. The duration from the first relapse to the onset was 5.5 months. (2)The positive rate of serum water channel aquaporin-4 antibody (AQP4-Ab) in the acute phase was 86.4%. The positive rate of cerebrospinal fluid (CSF) AQP4-Ab in the acute phase was 69.2%. The positive rate of autoimmune antibodies was 72.7%. There was a remarkable difference (Z=-12.632, P=0.000) in serum AQP4-Ab titer levels between with the acute and remission period (median titer of 1∶244.78 to 1∶139.63). There was a remarkable difference (Z=-20.161, P=0.000) in geometric mean of serum AQP4-Ab titer levels between with CSF AQP4-Ab positive (1∶289.8) and negative (1∶36.2). (3)63.6% of the uLETM patients had 10-15 contiguous segments, 31.8% had 16-19 contiguous segments and 4.5% had whole spinal cord affected. 72.7% of the lesions of uLETM were sliver. The detection rate of optic nerve lesion by MRI was 63.6% and brain sliver lesions was seen in 63.6% of the patients.(4) All patients improved after treatment with high-dose glucocorticoids (GCs) in the acute phase. 15 cases treated with long-term oral administration of low-dose GCs in remission stage of NMOSD. 6 cases treated with mycophenolate mofetil. 1 case treated with intravenous immunoglobulins. Conclusions: NMOSD with uLETM is predominantly seen in young woman. The high risk period of relapse is 5.5 months after the onset. A high portion of NMOSD patients with uLETM have serum and CSF AQP4-Ab in acute phase. The therapy of GCs is recommended in acute phase. Combination of GCs with immunosuppressant can achieve stable and satisfactory effect in remission period of NMOSD.