Coleman Bre'Ana, Sawhney Kiranpreet, LaPenna Paul
Neurology, Edward Via College of Osteopathic Medicine, Spartanburg, USA.
Neurology, Bon Secours St. Francis Downtown, Greenville, USA.
Cureus. 2024 Apr 16;16(4):e58406. doi: 10.7759/cureus.58406. eCollection 2024 Apr.
Anti-leucine-rich glioma-inactivated protein 1 (anti-LGI1) limbic encephalitis is a rare autoimmune neurologic disorder with antibodies against LGI1. It was first recognized as a disease in 2010 and represents the second most common cause of autoimmune encephalitis. Clinically, it is characterized by subacute changes in cognition, memory, and behavior, associated with hyponatremia and faciobrachial dystonic seizures (FBDS). This report discusses a unique onset of anti-LGI1 limbic encephalitis where an elderly female presented with symptoms of new-onset panic attacks and rhythmic facial movements for one week. She was then admitted to neurology for further serum, cerebrospinal fluid(CSF), and lab testing. She was eventually found to be positive for antibodies against LGI1 voltage-gated potassium channels, which confirmed the diagnosis of limbic encephalitis. The quick recognition of symptoms and escalation of management allowed the patient to experience drastic improvements after the initiation of steroids, immunotherapy, and lacosamide. Since anti-LGI1 limbic encephalitis is underdiagnosed, it can lead to irreversible long-term cognitive sequelae (i.e., memory deficits). Thus, awareness of the typically associated findings of FBDS, cognitive disturbances, psychiatric changes, and hyponatremia can aid in early diagnosis and prompt treatment with immunotherapy, allowing for more favorable outcomes.
抗富含亮氨酸胶质瘤失活蛋白1(抗LGI1)边缘叶脑炎是一种罕见的自身免疫性神经系统疾病,体内存在抗LGI1抗体。它于2010年首次被确认为一种疾病,是自身免疫性脑炎的第二大常见病因。临床上,其特征为认知、记忆和行为的亚急性改变,伴有低钠血症和面臂肌张力障碍性癫痫发作(FBDS)。本报告讨论了一例抗LGI1边缘叶脑炎的独特发病情况,一名老年女性出现新发惊恐发作和节律性面部运动症状达一周。随后她因进一步的血清、脑脊液(CSF)及实验室检查而入住神经内科。最终发现她抗LGI1电压门控钾通道抗体呈阳性,从而确诊为边缘叶脑炎。对症状的快速识别及管理的升级,使患者在开始使用类固醇、免疫疗法和拉科酰胺后病情大幅改善。由于抗LGI1边缘叶脑炎未得到充分诊断,可能会导致不可逆的长期认知后遗症(即记忆缺陷)。因此,了解FBDS、认知障碍、精神变化和低钠血症的典型相关表现有助于早期诊断,并通过免疫疗法进行及时治疗,从而获得更有利的结果。