Department of Neurology, Baoding No.1 Central Hospital, 320 Northern Great Wall Street, Hebei Province, 071000, Baoding, China.
Baoding City Key Laboratory of Neurological Diseases, Baoding, China.
BMC Neurol. 2022 Jul 27;22(1):281. doi: 10.1186/s12883-022-02797-w.
Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis, an autoimmune disorder, is characterized by faciobrachial dystonic seizures, epilepsy, memory deficits and altered mental status while hiccup is not commonly found in patients.
A 62-year-old male was presented with slurred speech, abnormal gait, faciobrachial dystonic seizures and impaired cognition. Besides, the hiccup was one of the initial symptoms. His brain magnetic resonance images (MRI) revealed multiple lesions with left caudate nucleus, putamen, insula and left hippocampus involvement. Because a diagnosis of antibody-related limbic encephalitis was suspected, studies including an autoimmune profile were done by cell-based assays. After anti-LGI1 antibodies were detected in both cerebrospinal fluid and serology, pulse methylprednisolone and intravenous immunoglobulin were started and hence hiccups disappeared along with other symptoms.
Clinicians should be aware that persistent hiccups might be one of the initial manifestations of LGI1 subtype of voltage-gated potassium channel complex antibody associated autoimmune encephalitis.
抗亮氨酸丰富胶质瘤失活 1 型(LGI1)脑炎是一种自身免疫性疾病,其特征为面臂肌张力障碍发作、癫痫、记忆障碍和精神状态改变,而呃逆在患者中并不常见。
一名 62 岁男性以言语不清、步态异常、面臂肌张力障碍发作和认知障碍就诊。此外,呃逆是首发症状之一。他的脑部磁共振成像(MRI)显示多个病变,涉及左侧尾状核、壳核、脑岛和左侧海马。由于怀疑为抗体相关边缘性脑炎,通过细胞基础检测进行了自身免疫谱等研究。在脑脊液和血清中均检测到抗 LGI1 抗体后,开始使用脉冲甲基泼尼松龙和静脉注射免疫球蛋白,呃逆和其他症状随之消失。
临床医生应意识到持续性呃逆可能是电压门控钾通道复合物抗体相关自身免疫性脑炎中 LGI1 亚型的初始表现之一。