Marasakatla S, Gupta M, Ramakrishnan S, Kulkarni G B
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.
J Postgrad Med. 2025 Apr 1;71(2):101-103. doi: 10.4103/jpgm.jpgm_756_24. Epub 2025 Jun 9.
Thunderclap headaches and multifocal cerebral artery constrictions characterize reversible cerebral vasoconstrictive syndrome (RCVS). Leucine-rich glioma-inactivated 1 (LGI-1) autoimmune encephalitis (AE) presents as limbic encephalitis, hyponatremia, and faciobrachial dystonic seizures. Their unusual presentation concurrently is unknown. We describe a rare case of LGI-1 AE with RCVS. A 31-year-old lady presented with acute onset visual loss and encephalopathy on the background of sleep behavioral symptoms. Retrospectively, the patient complained of having muscle twitching, and mood changes. Her blood pressure was high (220/120 mm Hg). Blood investigations revealed hyponatremia and positivity for LGI-1+ and anti-amphiphysin 1+ antibodies. Neuroimaging initially showed features of RCVS. The cerebrospinal fluid study was unremarkable. Electromyography showed florid fasciculations with myokymic discharges. She was treated with steroids and responded to immunotherapy (Azathioprine). She maintained well into follow-up. AE is a great mimicker. Knowledge about atypical presentations is important for guiding treatment and further clinical course.
霹雳样头痛和多灶性脑动脉狭窄是可逆性脑血管收缩综合征（RCVS）的特征。富含亮氨酸的胶质瘤失活1（LGI-1）自身免疫性脑炎（AE）表现为边缘叶脑炎、低钠血症和面臂肌张力障碍性癫痫发作。它们同时出现的不寻常表现尚不清楚。我们描述了一例罕见的LGI-1 AE合并RCVS病例。一名31岁女性在睡眠行为症状的背景下出现急性视力丧失和脑病。回顾性地看，患者抱怨有肌肉抽搐和情绪变化。她的血压很高（220/120毫米汞柱）。血液检查显示低钠血症以及LGI-1+和抗 amphiphysin 1+抗体呈阳性。神经影像学最初显示出RCVS的特征。脑脊液检查无异常。肌电图显示有明显的肌束震颤和肌阵发放电。她接受了类固醇治疗并对免疫疗法（硫唑嘌呤）有反应。随访期间她情况良好。AE是一种很容易造成误诊的疾病。了解非典型表现对于指导治疗和进一步的临床病程很重要。
