Department of Neurology, Govind Ballabh Pant Postgraduate Institute of Medical Education and Research, New Delhi, India.
Department of Neurology, Govind Ballabh Pant Postgraduate Institute of Medical Education and Research, New Delhi, India,
Eur Neurol. 2021;84(2):124-128. doi: 10.1159/000514106. Epub 2021 Mar 22.
Autoimmune encephalitis (AIE) constitutes an important treatable cause of movement disorders. We aimed to highlight the spectrum of movement disorder and other salient features of AIE patients diagnosed at our tertiary care centre and describe their clinical symptoms, diagnostic approach, treatment, and outcome. We evaluated 11 patients who presented with movement disorder in association with AIE at our centre. Various abnormal movements observed were tremor, dyskinesias, stereotypy, dystonia, ataxia, asterixis, myoclonus, and parkinsonism. Antibodies were detected against NMDAR (n = 3), LGI-1 (n = 2), GAD-65 (n = 1), CASPR-2 (n = 1), Sox-1 (n = 1), Yo (n = 1), and thyroid peroxidase (n = 1). One patient was diagnosed with opsoclonus myoclonus syndrome associated with the suspected neuroblastic tumour. Six patients responded well to first-line immunotherapy (intravenous immunoglobulins or steroid or both). Three patients with anti-NMDAR antibodies received second-line therapy consisting of rituximab. Movement disorder is one of the most consistent features of AIE. Understanding of the ever-expanding spectrum of antibodies associated with movement disorders helps in the early diagnosis and better management of patients of autoimmune movement disorder.
自身免疫性脑炎(autoimmune encephalitis,AIE)是一种重要的可治疗运动障碍的病因。我们旨在强调在我们的三级护理中心诊断出的 AIE 患者的运动障碍谱和其他显著特征,并描述他们的临床症状、诊断方法、治疗和结局。我们评估了在我们中心以运动障碍为表现的 11 例 AIE 患者。观察到的各种异常运动包括震颤、运动障碍、刻板动作、肌张力障碍、共济失调、扑翼样震颤、肌阵挛和帕金森病。共检测到针对 NMDAR(n=3)、LGI-1(n=2)、GAD-65(n=1)、CASPR-2(n=1)、Sox-1(n=1)、Yo(n=1)和甲状腺过氧化物酶(n=1)的抗体。1 例患者被诊断为与可疑神经母细胞瘤相关的眼阵挛-肌阵挛综合征。6 例患者对一线免疫治疗(静脉注射免疫球蛋白或皮质类固醇或两者)反应良好。3 例抗 NMDAR 抗体患者接受了包括利妥昔单抗在内的二线治疗。运动障碍是 AIE 最一致的特征之一。对与运动障碍相关的不断扩大的抗体谱的理解有助于早期诊断和更好地管理自身免疫性运动障碍患者。