刘玮,
孙路,
王莹,
胡雅娉,
杨林^,
大连医科大学附属第一医院 神经内科，辽宁 大连 116011

详细信息 作者简介: 刘 玮（1987-），女，主治医师。E-mail：www.weiweiforever@163.com





通讯作者: 杨 林，副主任技师。E-mail：sdf-yl@163.com 中图分类号: R741


摘要:目的探讨以面臂肌张力障碍发作（FBDS）为主要表现的LGI1抗体相关脑炎的脑电及影像学特点。方法收集2010年6月至2021年4月在大连医科大学附属第一医院神经内科确诊为以FBDS为主要表现的LGI1抗体相关脑炎的5例患者，均为女性，年龄21～68岁。对5例患者的临床表现、实验室检查、脑电图、影像学检查、治疗及预后等临床资料进行分析总结。结果5例患者均表现为FBDS，其中4例仅出现FBDS，1例表现为颞叶内侧癫痫样发作合并FBDS；5例均伴有认知功能障碍和焦虑抑郁表现，3例合并睡眠障碍，4例合并低钠血症。5例患者脑脊液、血清LGI1抗体滴度均阳性。5例中2例颅脑MRI示一侧基底节区（主要累及尾状核头、豆状核）异常信号；5例患者共监测到53次FBDS，发作期脑电表现为发作前或发作时全导电衰减伴广泛性中-高波幅多位相超低频（＜0.5 Hz）慢波叠加大量肌电伪差，其中47次为发作前电衰减，6次为发作时电衰减。结论FBDS是LGI1抗体相关脑炎的特异性癫痫发作类型，发作期脑电表现为全导电衰减伴广泛性中-高波幅多位相超低频（＜0.5 Hz）慢波叠加大量肌电伪差，结合影像学分析，考虑放电起源来自于皮层下深部基底节区。
关键词: LGI1抗体相关脑炎/
FBDS/
全导电衰减/
广泛性多位相超低频慢波


Abstract:ObjectiveTo explore the clinical,electroclinical and imaging features of LGI1 antibody-associated encephalitis with faciobrachial dystonic seizure (FBDS) as the main manifestation. MethodsClinical data of 5 patients with LGI1 antibody related encephalitis with FBDs as the main manifestation were collected from the Department of Neurology, the First Affiliated Hospital of Dalian Medical University from June 2010 to April 2021. All the patients were female, aged 21-68 years. The clinical manifestation, laboratory examination, EEG, imaging examination, treatment and prognosis of the 5 patients were analyzed and summarized. ResultsAll 5 patients demonstrated FBDS, of whom4 patients presented only FBDS, and 1 patient had MTLE-like seizure combined with FBDS. All 5 patients were associated with cognitive impairment and psychiatric symptoms, 3 patients were complicated with sleep disorder and 4 patients with hyponatremia. The titers of LGI1 antibody in cerebrospinal fluid and serum of 5 patients were all measured as positive. Among the 5 cases, 2 cases showed abnormal signals in the unilateral basal ganglia (mainly involving head of caudate nucleus and lentiform nucleus) on MRI. Totally, 53 episodes of FBDS were detected in the 5 patients. Ictal EEG showed full conductive attenuation before or during seizures with extensive medium-high amplitude and multiphase ultra-low frequency slow waves (frequency＜0.5 Hz) superimposed with a large number of EMG artifact. Preictal electrical attenuation (preictal 0.6-3.8 s) was found in 47 episodes. ConclusionsFBDS is a specific seizure type of LGI1 antibody-associated encephalitis. Ictal EEG shows full conductive attenuation with extensive medium-high amplitude and multi-phase ultra-low frequency slow wave (frequency＜0.5 Hz) with a large number of EMG artifact. Combined with imaging analysis, it is considered that the discharge originates from the deep subcortical basal ganglia.
Keywords:LGI1 antibody associated encephalitis/
FBDS/
full conductive attenuation/
extensive multi-phase ultra-low frequency slow wave

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