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抗LGI1抗体自身免疫性脑炎诊断的重要线索:一例报告

Important Clues for the Diagnosis of Anti-LGI1-Antibody Autoimmune Encephalitis: A Case Report.

作者信息

Abu-Abaa Mohammad, Chadalawada Sindhu, Jumaah Omar, Abubakar Malik, Landau Daniel

机构信息

Internal Medicine, Capital Health Regional Medical Center, Trenton, USA.

Neurology, Capital Health Regional Medical Center, Trenton, USA.

出版信息

Cureus. 2023 Jan 26;15(1):e34222. doi: 10.7759/cureus.34222. eCollection 2023 Jan.

Abstract

Anti-leucine-rich-glioma-inactivated 1 (LGI1) antibody autoimmune encephalitis is a rare autoimmune encephalitis. We present a 68-year-old female patient who initially presented with episodic confusion, hallucinations, behavioral changes, and unexplained hyponatremia. History was also remarkable for intermittent abnormal movement affecting the left upper extremity and face. She was initially thought to be suffering from dementia and was discharged home. However, progressive symptoms led to her second admission, where evidence of autonomic dysfunction with episodic bradycardia and persistent symptomatic orthostatic hypotension were evident. Generalized cortical hyperexcitability and subclinical seizures were seen. Diagnosis of LGI1 encephalitis was confirmed with a positive Anti-LGI1 antibody in the cerebrospinal fluid, and treatment with intravenous immunoglobulin and steroids improved her cognitive function. This case helps to highlight important features that should raise early clinical suspicion of LGI1 encephalitis, including unexplained progressive hyponatremia, autonomic dysfunction, and frequent refractory seizures. This can lead to earlier recognition of this condition, where earlier implementation of immunosuppressive therapy is linked to better clinical outcomes and brain structural preservation.

摘要

抗富含亮氨酸胶质瘤失活1(LGI1)抗体自身免疫性脑炎是一种罕见的自身免疫性脑炎。我们报告一名68岁女性患者,最初表现为发作性意识模糊、幻觉、行为改变及不明原因的低钠血症。病史中还存在累及左上肢和面部的间歇性异常运动。她最初被认为患有痴呆症并出院回家。然而,症状进展导致她再次入院,此时自主神经功能障碍的证据明显,表现为发作性心动过缓和持续性症状性直立性低血压。可见广泛性皮质兴奋性增高和亚临床癫痫发作。脑脊液中抗LGI1抗体阳性确诊为LGI1脑炎,静脉注射免疫球蛋白和类固醇治疗改善了她的认知功能。该病例有助于突出应引起对LGI1脑炎早期临床怀疑的重要特征,包括不明原因的进行性低钠血症、自主神经功能障碍和频繁的难治性癫痫发作。这可导致更早识别该疾病,早期实施免疫抑制治疗与更好的临床结局和脑结构保存相关。

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