Huang X, Xu F F, Qian H R, Lei X, Qi X K, Xia D Y
Department of Neurology, Beijing Luhe Hospital, Capital Medical University, Beijing 101100, China.
Zhonghua Yi Xue Za Zhi. 2018 Jun 5;98(21):1658-1663. doi: 10.3760/cma.j.issn.0376-2491.2018.21.008.
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. Twenty-two uLETM patients was recruited and retrospectively analyzed for general clinical characteristics, laboratory tests and MRI characteristics, as well as therapeutic. (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 (=-12.632, =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 (=-20.161, =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. 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.
分析伴有超长节段横贯性脊髓炎（uLETM）的视神经脊髓炎谱系障碍（NMOSD）的临床表现，以提高该疾病的诊断准确性。招募22例uLETM患者，对其一般临床特征、实验室检查、MRI特征及治疗情况进行回顾性分析。（1）男女比例为1∶6。发病年龄中位数为31岁。首次复发至发病的时间为5.5个月。（2）急性期血清水通道蛋白4抗体（AQP4-Ab）阳性率为86.4%。急性期脑脊液（CSF）AQP4-Ab阳性率为69.2%。自身抗体阳性率为72.7%。急性期与缓解期血清AQP4-Ab滴度水平有显著差异（=-12.632，=0.000）（中位数滴度从1∶244.78至1∶139.63）。CSF AQP4-Ab阳性（1∶289.8）与阴性（1∶36.2）患者血清AQP4-Ab滴度几何均值有显著差异（=-20.161，=0.000）。（3）63.6%的uLETM患者有10 - 15个连续节段受累，31.8%有16 - 19个连续节段受累，4.5%全脊髓受累。uLETM病变72.7%呈条片状。MRI检测视神经病变的发生率为63.6%，63.6%的患者有脑内条片状病变。（4）所有患者急性期经大剂量糖皮质激素（GCs）治疗后病情改善。15例在NMOSD缓解期接受长期小剂量GCs口服治疗。6例接受霉酚酸酯治疗。1例接受静脉注射免疫球蛋白治疗。伴有uLETM的NMOSD主要见于年轻女性。复发高危期为发病后5.5个月。大部分伴有uLETM的NMOSD患者急性期血清及CSF AQP4-Ab阳性。急性期推荐使用GCs治疗。GCs与免疫抑制剂联合应用在NMOSD缓解期可取得稳定且满意的效果。
