表现为超长节段脊髓炎的视神经脊髓炎谱系疾病的临床特征

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

目的分析表现为超长节段横贯性脊髓炎（uLETM）的视神经脊髓炎谱系疾病（NMOSD）的临床特点，以提高对本病的认识。方法回顾性分析2009年7月29日至2015年12月3113海军总医院确诊的22例以uLETM为主要表现的NMOSD临床表现、实验室特点、MRI特征及诊治经过。结果（1）一般临床特点：男女比例约1：6。首次发病中位年龄31岁。第一次复发距首次发病的中位时间间隔5．5个月。（2）实验室特点：急性期血清水通道蛋白-4抗体（AQP4-Ab）阳性率86．4％，急性期脑脊液（CSF）AQP4-Ab阳性率69．2％，血清其他自身免疫抗体阳性率72．7％。急性期血清AQP4．Ab抗体滴度（几何均数为1：244．78）高于缓解期（几何均数为1：139．63），差异具有统计学意义（Z=-12．632，P=0．000）。CSFAQP4-Ab阳性患者（几何均数1：289．8）的血清AQP4-Ab抗体滴度明显高于CSFAQP4-Ab阴性患者（几何均数1：36．2），差异具有统计学意义（Z=-20．161，P=0．000）。（3）影像学特点：63．6％累及10—15个椎体节段，31．8％累及16—19个椎体节段，4．5％累及全脊髓节段。72．7％的脊髓MRI病灶形态呈条片样。63．6％合并视神经损伤，63．6％合并颅脑斑片样脱髓鞘病灶。（4）治疗：急性期均经大剂量糖皮质激素（GCs）冲击治疗。缓解期：15例口服小剂量GCs维持治疗，6例联合吗替麦考酚酯治疗，1例联合丙种球蛋白序贯治疗。结论表现为uLETM的NMOSD好发于青年女性，年复发率较高，首次发病后5．5个月是复发的高危时期。急性期血清及CSFAQP4-Ab检出率较高，采用GCs治疗敏感性较好，缓解期小剂量GCs联合免疫抑制剂治疗效果稳定。

Objective To analyze the clinical presentations of neuromyelitis optica spectrum disorders （NMOSD） with uhra-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. Coneltmions 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 NMOSD. t can achieve stable and satisfactory effect in remission period of