成人MOG抗体相关性脑脊髓炎患者15例临床特点分析

目的 探讨成人髓鞘少突胶质细胞糖蛋白（MOG）抗体相关性脑脊髓炎（MOG-EM）患者的临床特点。方法 回顾性分析15例成人MOG-EM患者的临床症状、影像学特点、实验室检查、预后及随访等情况。结果 15例患者中男7例、女8例,起病年龄（39.0±14.7）岁。临床症状以视力下降最多见（7例,7/15）,其次为肢体麻木或肢体瘫痪（5例,5/15）,部分患者有构音不清、视物重影、行走不稳等脑干、小脑症状或意识障碍、癫痫发作、记忆力下降等急性播散性脑脊髓炎样症状。15例患者行头颅MRI检查,13例（13/15）显示有异常病灶,以大脑皮层及皮层下白质（8例,8/13）、桥脑（4例,4/13）受累多见,丘脑、小脑、胼胝体亦可见受累。9例患者行全脊髓MRI检查,3例（3/9）有异常病灶、均累及颈髓,1例（1/3）累及胸髓。15例患者血清MOG-IgG均阳性,其中11例行脑脊液MOG-IgG检测,5例（5/11） 阳性。8例（8/15） 患者临床复发。10例患者复查血清MOG-IgG,5例（5/10）抗体转阴,其中4例（4/5）无复发;5例（5/10）抗体呈持续阳性,均复发。所有患者经甲泼尼龙及Ig治疗,1例加用吗替麦考酚酯治疗,预后均良好。结论 成人MOG-EM以视神经炎表现常见,大脑皮层、皮层下白质、脑桥易受累,预后较好,临床复发率较高,血清MOG-IgG持续阳性者易复发。

Clinical characteristics of 15 adult cases of MOG-IgG-associated encephalomyelitis

Objective To investigate the clinical characteristics of 15 adult patients with myelin oligodendrocyte glycoprotein （MOG） immunoglobulin-G （IgG）-associated encephalomyelitis （MOG-EM）. Methods The clinical symptoms, MRI features, laboratory examination, clinical prognosis and follow-up of 15 MOG-EM patients were retrospectively analyzed. Results Among 15 patients, 7 cases were male and 8 female. The average age of onset was （39.0±14.7） years. The most common clinical symptoms were visual impairment（7/15）, followed by limb numbness and paralysis （5/15）. Partial patients presented with relevant symptoms of the brainstem and cerebellum （dysarthria, double vision and unstable walking） or relevant signs of acute disseminated encephalomyelitis, such as consciousness disorder, seizure and memory loss, etc. MRI results detected abnormal lesions in 13 cases （13/15） including 8 cases of cortical/subcortical white matter involvement （8/13） and 4 cases of pontile involvement （4/13）. The thalamus, cerebellum and corpus callosum were also involved. Nine patients received MRI of the whole spinal cord. Among them, 3 cases presented with abnormal lesions, all of which were involved with the cervical spinal cord and 1 case of thoracic spinal cord （1/3）. All 15 patients were tested positive for serum MOG-IgG. Among 11 cases receiving detection of MOG-IgG in the cerebrospinal fluid, 5 cases were positive for MOG-IgG. Eight （8/15） patients recurred. Ten patients received repeated detection of serum MOG-IgG. Among them,5 cases turned negative and 4 of them did not recur. The remaining 5 cases remained positive and recurred. All patients were treated with methylprednisolone and Ig. One patient was supplemented with mycophenolate mofetil. Favorable clinical prognosis was obtained. Conclusions Adult patients with MOG-EM are primarily manifested with optic neuritis, which is involved with cerebral cortex, subcortical white matter and pons. Relatively favorable clinical prognosis can be obtained. The cliical recurrence rate is relatively high. Patients persistently positive for serum MOG-IgG are likely to recur.