视神经脊髓炎脑部瘤样脱髓鞘病变五例临床及影像学特征分析

Clinical and radiological characteristics in five cases of neuromyelitisoptica with tumefactive-like demyelinating lesions

目的探讨视神经脊髓炎（neuromyelitis optica，NMO）脑部瘤样脱髓鞘病变（tumefactive—like demyelinating lesions，TDLs）的临床及影像学特点。方法对2007年12月至2012年3月在中山大学附属第三医院神经科住院的5例脑部出现TDLs的NMO患者的临床、影像学特点、治疗及预后进行回顾性分析。结果5例患者中男性1例，女性4例，发病年龄17—44岁，平均32岁，TDLs出现的平均时间为首次发病后的36个月。5例患者均存在血清NMO．免疫球蛋白G（IgG）阳性，4例出现脑病症状（昏迷2例，抽搐1例，嗜睡1例，同向性偏盲1例）。行头颅MRI见TDLs均孤立生长，位于顶枕叶者2例，顶叶1例，小脑1例，颞叶1例；MRI增强4例未见TDLs强化，1例呈开环样强化。3例患者的TDLs病灶在行DWI及表观弥散系数检查时为混杂信号，表观弥散系数值均升高，2例出现明显占位效应及1例伴明显水肿。后经MRI随访1例TDLs消失，3例明显缩小，1例基本不变，3例伴空洞形成。全部患者经大剂量激素贯续冲击及丙种球蛋白治疗，均获得不同程度缓解。结论NMO脑部TDLs临床并不十分少见，多伴不同类型的脑病症状。TDLs急性期多不强化，伴广泛的血管源性水肿。NMO—IgG检测及头颅MRI检查对TDLs诊断有重要意义，对激素及丙种球蛋白治疗敏感。

Objective To analyze clinical and radiological characteristics in 5 cases of neuromyelitisoptica （NMO） with tumefactive-like demyelinating lesions （TDLs）. Methods Five NMO patients with TDLs hospitalized during November 2007 and March 2012 were retrospectively analyzed for clinical and magnetic resonance imaging （MRI） features, as well as therapeutic efficiency and prognoses. Results The male-to-female ratio is 1：4 and the range of onset ages is 15----44 years old. The median time when TDLs was developed was 36 months after the onset of NMO. The anti- aquaporin 4 was seropositive in all NMO patients with TDLs. Four patients had clinical manifestations of encephalopathy symptoms including coma （ n = 2）, seizures （ n = 1 ）, narcolepsy （ n = 1 ）, homonymous hemianopia （ n = 1 ）. All TDLs grew unilaterally. Two TDLs were located at parietal-occipital, 1 at parietal, 1 at the cerebellum and 1 at the temporal. On MRI, there was no enhanced signal in 4 TDLs, and there was open-ring-like enhanced signals in 1 case. Three TDLs revealed mixed signals intensities on difiusion-weighted images and an increase in the apparent difiusion coeficient values. On the subsequent MRIs, the sizes of 5 TDLs were markedly reduced in 3 cases, disappeared in 1 and another I case remained the same. Meanwhile, 3 lesions subsequently developed cavity forming. Five cases improved after treatment of high-dose corticosteroid and intravenous immune globulin. Conclusions TDLs is not uncommon in NMO. Encephalopathysymptoms are more frequently affected in NMO with TDLs. TDLs often accompany with extensive vasogenic edema, but without enhancement in acute stage. NMO-IgG antibody testing and brain MRI are important in the diagnosis of TDLs. The therapy of corticosteroid and intravenous immune globulin is recommended.