抗谷氨酸脱羧酶抗体阳性自身免疫性脑炎临床-电-影像学特征分析  

Clinical,electroclinical and imaging features in patients with anti-glutamate decarboxylase antibody-positive autoimmune encephalitis

作　　者：苗爱亮 王凌玲[1] 余年[1] 王迎新 葛剑青[1] MIAO Ai-liang;WANG Ling-ling;YU Nian(Department of Neurology,Affiliated Brain Hospial of Nanjing Medical University,Narjing 210029,China)

机构地区：[1]南京医科大学附属脑科医院神经内科,210029

出　　处：《临床神经病学杂志》2020年第5期339-343,共5页Journal of Clinical Neurology

基　　金：国家自然科学基•金青年项目(81501126);江苏省青年医学重点人才(QNRC2016053);南京医科大学附属脑科医院青年人才项目。

摘　　要：目的探讨抗谷氨酸脱羧酶(GAD)抗体阳性自身免疫性脑炎的临床-电-影像学特征。方法对本院自2015年5月至2020年5月期间明确诊断的5例抗GAD抗体阳性自身免疫性脑炎患者进行回顾性研究,分析临床症状学、EEG、影像学特征及其与预后之间关系。结果 5例患者中4位为女性,发病年龄分别为44岁(例1)、26岁(例2)、27岁(例3)和17岁(例14)。2例女性患者以新发难治性癫痫持续状态起病, 2例女性患者以癫痫频发起病。1例老年男性(例5),以反应迟钝起病,发病年龄为70岁。4例女性患者初次EEG背景为中-重度异常,明显差于男性患者。伴有双侧岛叶、内侧颞叶及海马异常信号的例1、例3和例4患者中观察到视频EEG存在明显的局灶性异常的不稳定性。例3起病3年时视频EEG清醒及睡眠期频见起源于左颞的临床下发作,发病4年时出现左上肢的皮质下肌阵挛。例4发病20个月时清醒及睡眠期频见起源右颞局灶性发作伴意识障碍和临床下发作。此3例患者均遗留癫痫发作及明显的记忆力障碍。例2和例5的病灶位于大脑中线附近,尽管例2以新发难治性癫痫持续状态起病,发病初期EEG背景重度异常,但经过治疗,未遗留癫痫发作及明显记忆力减退。例5经过免疫治疗,基本恢复正常。此2例患者预后较好。结论临床中首次出现癫痫发作的患者,且癫痫有频发的趋势或新发难治性癫痫持续状态,视频EEG中频见起源单侧或者双侧颞叶局灶性发作和/或临床下发作,建议筛查免疫因素。伴有双侧岛叶、内侧颞叶及海马受累的抗GAD抗体阳性的患者EEG局灶性异常存在明显不稳定性,这或许是抗GAD抗体阳性患者癫痫难治的原因之一。而且这类患者预后差,往往遗留癫痫及记忆力下降。Objective To determine the clinical,electroclinical and imaging features in patients with anti-glutamate decarboxylase(GAD) antibody-positive autoimmune encephalitis(AE). Methods Five patients with anti-GAD antibody-positive AE admitted to Nanjing Brain Hospital from May 2015 to May 2020 were retrospectively analyzed. The clinical characteristics and the features of video EEG and brain imaging were collected. And their relationship with the prognosis was also analyzed. Results Among the five patients, four of them were female. Their ages at the disease onset were 44 years old(case 1), 26 years old(case 2), 27 years old(case 3) and 17 years old(case 4), respectively. Two female patients were presented with new-onset refractory status epilepticus, and another 2 female patients were onset with frequent seizures. One oldly male patient was onset with unresponsive. Their EEG background was moderate and severe abnormal, which was relatively worse in the 4 female cases than that in the male case. The obviously unstable focal abnormality in video EEG was observed in case 1, case 3 and case 4 with abnormal signals in bilateral insular lobe, medial temporal lobe and hippocampus. For case 3, subclinical seizures originating from the left temporal lobe were frequently seen during awake and sleeping periods after 3 years of onset, and subcortical myoclonus of the left upper limb occurred after 4 years of onset. For case 4, focal attack with disturbance of consciousness and subclinical seizures originating from right temporal were found frequently both in awake and sleeping period after 20 months of onset. All the three patients above showed epilepsy and obvious memory impairment. The brain lesions in case 2 and case 5 were mainly located near the midline of the brain. Although case 2 was onset with new-onset refractory status epilepticus and severe abnormal EEG background, no epileptic seizures and significant memory loss were found after immunotherapy. Case 5 was recovered to normal after immunotherapy.The two patients above

关 键 词：抗谷氨酸脱羧酶抗体脑炎 临床特点 视频EEG 影像学 预后 

分 类 号：R742.9[医药卫生—神经病学与精神病学]