Ictal Asystole and Anti-N-Methyl-D-aspartate Receptor Antibody Encephalitis
Ictal Asystole and Anti-N-Methyl-D-aspartate Receptor Antibody Encephalitis
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DOI：
10.1542/peds.2010-2080
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发表时间：
2011-03-01
期刊：
影响因子：
8
通讯作者：
Wainwright, Mark S.
中科院分区：
文献类型：
--
作者：
Millichap, John J.;Goldstein, Joshua L.;Wainwright, Mark S.
抗 N-甲基-D-天冬氨酸受体 (NMDAR) 抗体脑炎是最近发现的一种自身免疫性疾病，在儿童中越来越受到重视。大多数病例发生在女孩和妇女中，可能是伴有卵巢畸胎瘤的副肿瘤。典型的临床特征包括神经精神症状、运动障碍、意识下降和自主神经不稳定。我们报告了第一例与颞叶癫痫发作相关的心搏停止病例，并强调需要仔细监测这种潜在致命的并发症。一名先前健康的 15 岁女孩出现局灶性癫痫发作和性格改变，并逐渐发展为烦躁、混乱和紧张症交替出现的时期。在脑脊液和血清中检测到抗NMDAR抗体。初次就诊后 26 天，出现新的癫痫发作，其特征是心动过缓和氧饱和度下降。连续视频脑电图监测捕获了 3 次癫痫发作，伴有左颞叶发作和相关的心搏停止。通过盆腔超声和计算机断层扫描诊断出卵巢畸胎瘤，随后进行手术切除，神经精神症状逐渐改善。从第 26 天开始使用苯巴比妥治疗可导致癫痫发作停止。然而，无症状的心动过缓和3秒的停顿仍在继续。第 46 天插入按需起搏器后，没有再发生心脏事件。该患者还接受了2个疗程的静脉注射免疫球蛋白治疗。 4 个月的门诊随访显示神经系统几乎完全恢复，并且没有心脏事件。据我们所知，发作性心搏停止以前并未被描述为抗 NMDAR 脑炎的并发症；对于这种新出现的儿科疾病来说，这是一种可以预防的死亡原因，这种疾病的症状千变万化，很容易被误诊。儿科 2011；127：e781-e786
Anti-N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis is a recently identified autoimmune disorder that is increasingly recognized in children. Most cases occur in girls and women and may be paraneoplastic with an associated ovarian teratoma. Characteristic clinical features include neuropsychiatric symptoms, dyskinesias, decreased consciousness, and autonomic instability. We report the first case of asystole associated with temporal lobe seizures in this disorder and highlight the need for careful monitoring for this potentially fatal complication. A 15-year-old previously healthy girl presented with focal seizures and personality changes that progressed to periods of agitation and confusion alternating with catatonia. Anti-NMDAR antibodies were detected in the cerebrospinal fluid and serum. Twenty-six days after initial presentation, new seizures developed characterized by bradycardia and oxygen desaturation. Continuous video-electroencephalogram monitoring captured 3 seizures with left-temporal onset and associated asystole. An ovarian teratoma was diagnosed by pelvic ultrasound and computed tomography, and surgical resection was followed by gradual improvement in her neuropsychiatric symptoms. Treatment with phenobarbital beginning on day 26 lead to the cessation of seizures. However, asymptomatic bradycardia and pauses of 3 seconds continued. After insertion of a demand pacemaker on day 46, there were no further cardiac events. The patient was also treated with 2 courses of intravenous immunoglobulin. Outpatient follow-up at 4 months revealed near-complete neurologic recovery and no cardiac events. To our knowledge, ictal asystole has not previously been described as a complication of anti-NMDAR encephalitis; it is a preventable cause of death in this emerging pediatric disorder, which presents with protean symptoms and is easily misdiagnosed. Pediatrics 2011;127:e781-e786