背景:自身免疫性脑炎(AIE)是一类相对较新描述的涉及中枢神经系统的免疫介导疾病,具有广泛的临床表现,从相对轻度或隐匿的认知障碍发作到更复杂形式的脑病并伴有医学难治性癫痫发作。伴有神经精神症状和认知或记忆障碍的单灶性或多灶性癫痫发作提示AIE发作时的临床特征。
方法:一个六岁男孩出现重复性局灶性癫痫发作,慢慢进步的情感责任,和注意力缺陷/多动障碍样症状。癫痫发作类型在临床过程中有所不同,有时会出现集群或状态。在癫痫发作聚集/状态期间进行的MRI显示运动信号异常。我们成功地用大剂量静脉注射甲基强的松龙治疗了患者。脑脊液分析显示细胞增多,针对N-甲基-d-天冬氨酸型谷氨酸受体亚基和颗粒酶B的N末端抗体明显升高。
结论:我们报告了一例癫痫发作灶移动,MRI表现异常。尽管精神症状的发作缓慢发展为AIE的非典型症状,对免疫疗法的反应,脑脊液细胞增多症,和自身抗体都表明AIE。因此,我们建议癫痫发作灶的移动和MRI信号异常可能是AIE的发现。
BACKGROUND: Autoimmune encephalitis (AIE) is a relatively newly described category of immune-mediated diseases involving the central nervous system with a wide spectrum of clinical presentations, ranging from relatively mild or insidious onset of cognitive impairment to more complex forms of encephalopathy with medically refractory seizures. Single or multifocal seizures accompanied by neuropsychiatric symptoms and cognitive or memory impairments are suggestive of clinical features at AIE onset.
METHODS: A six-year-old boy presented with repetitive focal seizures, slowly progressive emotional liability, and attention-deficit/hyperactivity disorder-like symptoms. Seizure types varied during the clinical course, sometimes emerging as clusters or statuses. MRI performed during seizure clustering/status revealed moving signal abnormalities. We successfully treated the patient with high-dose intravenous methylprednisolone. Cerebrospinal fluid analysis revealed pleocytosis and marked elevation of antibodies against N-terminals of N-methyl-d-aspartate type glutamate receptor subunits and granzyme B.
CONCLUSIONS: We report a
case of moving seizure foci with abnormal MRI findings. Although the onset of psychiatric symptoms slowly progressed to those atypical for AIE, responsiveness to immunotherapy, cerebrospinal fluid pleocytosis, and autoantibodies all indicated AIE. We thus suggest that moving seizure foci and abnormal MRI signals may be findings of AIE.