导致致命性喉痉挛的抗N-甲基-M-天冬氨酸受体脑炎一例

Ictal Iaryngospasm as a main feature in a patient with anti-NMDA receptor encephalitis

抗N-甲基-M-天冬氨酸受体脑炎是一种少见的自身免疫性全脑炎。本病例报道的1例抗N-甲基-M-天冬氨酸受体脑炎的患者伴有严重的发作性喉痉挛。17岁男孩因难治性精神行为症状和认知能力下降来诊。脑组织磁共振平扫和DWI未见异常。血清和脑脊液中抗N-甲基-M-天冬氨酸受体抗体阳性,证实了抗N-甲基-M-天冬氨酸受体脑炎的诊断。住院期间该患者出现了严重的发作性喉痉挛,导致气管插管、气管切开,反复应用免疫球蛋白和大剂量激素冲击治疗。经治疗患者好转出院并接受门诊定期随访。报道该病例的目的旨在提高人们的认识,抗N-甲基-M-天冬氨酸受体脑炎患者可能表现出致命的发作性喉痉挛,需要紧急和积极的气道管理及免疫治疗。

Anti-N-methyl-aspartate receptor encephalitis(anti-NMDARE)is a rare autoimmune panencephalitis.We report the case of a patient with anti-NMDARE with severe ictal laryngospasm.A 17-year old boy was referred to our neurological intensive care unit for refractory behavioural symptoms and cognitive decline.Brain magnetic resonance imaging was normal.Screening for antineuronal antibodies showed positive results for anti-NMDA in serum and cerebrospinal fluid,which confirmed the diagnosis of anti-NMDARE.During his admission,he developed severe ictal laryngospasm resulting in intubation,tracheostomy,and repeated courses of intravenous immunoglobulin and methylprednisolone.The patient then made an uneventful recovery and was discharged to outpatient follow-up.Our report intends to raise awareness that patients with anti-NMDARE may manifest fatal ictal laryngospasm,requiring urgent and aggressive management.