抗AQP4抗体对脑干临床孤立综合征转归的影响

Value of anti- aquaporin 4 antibody in conversion of brainstem clinically isolated syndrome

目的研究抗水通道蛋白4（AQP4）抗体对以脑干损伤为首发表现的临床孤立综合征（CIS）患者疾病转归的影响。方法回顾性分析中山大学附属第三医院多发性硬化中心资料库自2006年7月至2010年12月登记的31例脑干临床孤立综合征（BCIS）患者的病例资料。根据血清抗AQP4抗体情况，将入组患者分为2组：抗AQP4抗体（＋）BCIS组（n=14）及抗AQP4抗体（-）BCIS组（n=17）。比较抗AQP4抗体（＋）BCIS组与抗AQP4抗体（-）BCIS组患者在临床症状、影像学、脑脊液等方面的异同点，分析2组患者临床转归的差异。结果31例入组患者平均随访（36.10±18.94）月，9例（64.29%）抗AQP4抗体（＋）BCIS组患者转化为视神经脊髓炎（NMO），而仅2例（11.76%）抗AQP4抗体（-）BCIS组患者转化为NMO，转化率差异有统计学意义（P〈0.05）。抗AQP4抗体（＋）BCIS组患者消化系统相关性脑干症状发生率（71.43% vs 23.53%）、脑脊液IgG指数（0.68±0.43 vs 0.47±0.19）、末次随访时的扩展残疾状况评分量表（EDSS）评分（4.64±0.92 vs 2.71±0.81）较抗AQP4抗体（-）BCIS组显著增高，差异均有统计学意义（P〈0.05）。影像学方面：抗AQP4抗体（＋）BCIS组患者延髓病灶（78.57% vs 35.29%）及脑干背侧病灶（78.57% vs 41.17%）较抗AQP4抗体（-）BCIS组明显增多，差异均有统计学意义（P〈0.05）。结论血清抗AQP4抗体检测对预测BCIS的临床转归有一定价值。

Objective Brainstem clinically isolated syndrome （BCIS） may herald multiple sclerosis （MS） or neuromyelitis （NMO）, or it may occur as an isolated syndrome. However, the role of anti-aquaporin 4 antibodies in the conversion of BCIS to NMO remains uncertain. Methods Thirty-one BCIS patients hospitalized in our hospital from July 2006 to December 2010 were chosen in our study; their clinical data were retrospectively analyzed; according to the presence of anti-AQP4 antibodies, they divided into two groups： anti-AQP4（＋）BCIS group （n=14） and anti-AQP4（-）BCIS group （n=17）. The differences of clinical manifestations, imaging features and cerebrospinal fluid level between the two groups were compared and the conversion of patients from the two groups was analyzed. Results Nine （64.29%） anti-AQP4 （＋）BCIS patients converted to NMO, while only two （11.76%） anti-AQP4 （-）BCIS patients progressed to NMO, over a mean follow-up period of 36.10±18.94 months; significant difference of conversion rate was noted between the two groups （P〈0.05）. Anti-AQP4（＋）BCIS patients demonstrated a higher immunogamm globulin （IgG） index （0.68±0.43 vs. 0.47±0.19, P=0.018） and Kurtzke Expanded Disability Status Scale （EDSS） scores at the last visit of follow-up （4.64±0.92 vs. 2.71±0.81, P=0.000） than anti-AQP4 （-）BCIS patients. Gastroenterology-related brainstem symptoms occurred more frequently in anti-AQP4（＋）BCIS patients than that in anti-AQP4（-）BCIS patients （71.43% vs. 23.53%, P=0.001）. In brainstem MR imaging, the anti-AQP4（＋）BCIS patients were observed having medulla-predominant involvement in the sagittal view （78.57%） and dorsal-predominant involvement in the axial view （78.57%）, which were significantly different as compared with those in the anti-AQP4（-） BCIS patients （35.29% and 41.17%, P〈0.05）. Conclusion anti-AQP4（＋）BCIS may represent an early, isolated syndrome of NMO spectrum disorder.