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- W3024588042 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.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31日海军总医院确诊的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)。CSF AQP4-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CSF AQP4-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CSF AQP4-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。." @default.
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- W3024588042 date "2018-06-05" @default.
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- W3024588042 title "[Clinical presentations of neuromyelitis optica spectrum disorders with ultra-longitudinally extensive transverse myelitis]." @default.
- W3024588042 doi "https://doi.org/10.3760/cma.j.issn.0376-2491.2018.21.008" @default.
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