摘要目的 分析视神经脊髓炎谱系疾病(NMOSD)伴有脊髓空洞症(SML)样表现的临床特点.方法 回顾性分析2008年6月至2016年8月中山大学附属第三医院的7例NMOSD伴SML样表现患者临床表现、实验室指标、影像学表现等.结果 7例患者中女6例,男1例,年龄27~67岁,病程3~12年,复发次数2~7次,脑脊液寡克隆区带(CSF OCB)均阴性,5例血视神经脊髓炎IgG(NMO-IgG)抗体阳性,以肢体感觉、运动及二便功能障碍为主要表现,尤其疼痛明显,脊髓空洞样表现主要出现在C4~T10,空洞长2~6 mm,直径0.8~3.6 mm,脑叶受累5例,长节段脊髓炎受累6例.结论 NMOSD伴有SML样表现患者女性多见,临床表现以肢体疼痛为著,病情易进展,复发率、致残率高,血NMO-IgG抗体阳性多见,脊髓空洞主要位于脊髓下颈段上胸段,以脑叶、长节段脊髓炎受累常见.

abstractsObjective To analyze the clinical characteristics of neuromyelitis optica spectrum disorders (NMOSD) associated with syringomyelia (SML).Methods The clinical manifestations,laboratory parameters and imaging findings of 7 patients with NMOSD associated with SML during June 2008 to August 2016 from The Third Affiliated Hospital of Sun Yat-sen University were retrospectively analyzed.Results Six patients were female and 1 was male,with ages ranging from 27-67 years,the course of the disease was 3-12 years,recurrence was 2-7 times,all the patients were cerebrospinal fluid oligoclonal bands (CSF OCB) negative,5 patients were positive for aquaporin-4-antibody/NMO-IgG.Sensory and motor dysfunction,bowel or bladder dysfunction were the common clinical manifestation,and pain was a significant symptom.Syringomyelia was predominantly located in C4-T10,cavity length 2-6 mm,diameter 0.8-3.6 mm;5 had lobes involvement and 6 had long segmental spinal cord involvement.Conclusions NMOSD associated with SML had a high frequency of occurrence in female,with the clinical characteristics of limb pain,easy progression,high recurrence rate and disability rate.The anti AQP4 antibody were commonly seen in these patients.Syringomyelia was predominantly located in the lower cervical and upper thoracic spinal cord,the lobar and longitudinally extensive transverse myelitis were frequently found in patients with NMOSD associated with SML.