超长节段横贯性脊髓炎临床特点分析

目的 分析超长节段横贯性脊髓炎的临床特点。方法 对2009年9月至2011年3月在中日友好医院神经内科住院的4例首次发病的超长节段横贯性脊髓炎患者的临床、影像学特点以及治疗和预后进行回顾性分析。结果 男1例,女3例,发病年龄为29 ～ 33岁。本病多以肢体瘫痪伴尿便障碍为首发,临床表现包括视力异常、运动、感觉以及尿便障碍等视神经和脊髓受累症状和体征,3例患者血尿酸降低,血清白身免疫抗体谱及NMO-IgG可阳性,头颅MRI无典型脱髓鞘病灶,脊髓MRI表现为长T1,长T2病灶几乎累及脊髓全长,可伴脊髓肿胀,其中2例患者确诊为干燥综合征伴超长节段横贾性脊髓炎。4例患者均经大剂量激素、丙种球蛋白以及免疫抑制剂治疗,3例获得不同程度缓解,1例死亡。结论 超长节段横贯性脊髓炎好发于青年女性,以脊髓受累多见,可合并视神经受累及其他自身免疫性疾病,颅内多无病灶,对激素等免疫抑制剂敏感。

Clinical features of ultra longitudinally extensive transverse myelitis

Objective To analyze the clinical features of ultra longitudinally extensive transverse myelitis (uLETM). Methods Four first-onset uLETM patients hospitalized during September 2009 and March 2011 were recruited and retrospectively analyzed for clinical and MRI ( magnetic resonance imaging)features, as well as therapeutic profiles and prognoses. Results The male-to-female ratio was 1∶3 and the age-of-onset 29-33 years old. Extremity paralysis and intrinsic sphincter disorders were initially observed. The clinical manifestations include visual, motor, sense and intrinsic sphincter disorders caused by optic nerve and spinal cord lesions. Uric acid decreased in 3 cases. Seropositivity for autoimmune antibody spectrum and NMO-IgG ( neuromyelitis optica-immunoglobulin G) was found in some patients. Spinal MRI showed overall hypointense T1 and hyperintense T2 lesions in spinal cord with partial swelling and negative in brain MRI. Sjogren's syndrome associated with uLETM was diagnosed in 2 patients. Three cases improved after treatment with high-dose corticosteroids, intravenous immunoglobulin and other immunosuppressive agents. One patient died. Conclusion uLETM is commonly found in young women. Spinal cord is frequently affected. And it may occur concurrently with optical abnormalities and other autoimmune diseases, Intracranial parenchyma is rarely affected. The therapy of corticosteroids is recommended.