Anti-LGI1 Autoimmune Limbic Encephalitis: An Easy-To-Miss Diagnosis.

Q3 Medicine
European journal of case reports in internal medicine Pub Date : 2024-11-19 eCollection Date: 2024-01-01 DOI:10.12890/2024_005025
Carolina Freitas Henriques, Rui Fernandes, Leonor Neves, Rubina Miranda, Duarte Noronha, Teresa Carolina Aguiar, Teresa Faria
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引用次数: 0

Abstract

Background: Autoimmune limbic encephalitis (ALE) is a rare inflammatory disorder characterised by a subacute onset, usually within weeks. The presence of multiple neuropsychiatric symptoms such as seizures, short-term memory deficits, anxiety and depression often leads to misdiagnosis as another medical condition, contributing to poor prognosis and reduced long-term survival.

Case description: A 60-year-old man, with no chronic illnesses, presented at the emergency department with daily episodes of palpitations, shivering, piloerection and a sense of impending doom lasting two months. Initially diagnosed with anxiety disorder and treated with venlafaxine 50 mg daily, he showed no improvement and developed memory loss. Hospitalised three months later, he exhibited both temporal and spatial disorientation, along with short-term memory loss. Key findings included elevated serum sedimentation rate, hyponatraemia, increased cerebrospinal fluid (CSF) protein levels and cranial magnetic resonance imaging evidence of bilateral temporal intra-parenchymal lesions, suggesting limbic encephalitis. After ruling out alternative diagnoses, screening of autoantibodies in the CSF was requested, which was positive for anti-LGI1 antibodies. The diagnosis of anti-LGI1 ALE was assumed, and treatment was initiated with significant clinical and imaging improvement.

Conclusions: ALE's broad clinical spectrum contributes to underdiagnosis. Therefore, in patients with new onset of neuropsychiatric symptoms and no prior psychiatric history, ALE should be considered, as prompt diagnosis and treatment are pivotal to achieve a good prognosis.

Learning points: Autoimmune limbic encephalitis is a rare inflammatory neurological disease that affects the limbic system particularly the hippocampus, leading to memory impairment and neuropsychiatric symptoms.Due to its wide range of neuropsychiatric symptoms, the diagnosis of autoimmune limbic encephalitis may go unnoticed, leading to misdiagnosis as another medical disorder.Early diagnosis is essential to prevent potential neurological sequelae through appropriate treatment.

抗 LGI1 自身免疫性边缘脑炎:容易被忽视的诊断
背景:自身免疫性边缘脑炎(ALE)是一种罕见的炎症性疾病,以亚急性发作为特征,通常在数周内发作。出现多种神经精神症状,如癫痫发作、短期记忆缺陷、焦虑和抑郁,往往导致误诊为另一种疾病,导致预后不良和长期生存时间缩短。病例描述:一名60岁男性,无慢性疾病,每日出现心悸、颤抖、勃起和即将到来的末日感,持续两个月。他最初被诊断为焦虑症,每天服用50毫克文拉法辛治疗,但没有任何改善,还出现了记忆力减退。三个月后住院,他表现出时间和空间定向障碍,以及短期记忆丧失。主要发现包括血清沉降率升高、低钠血症、脑脊液(CSF)蛋白水平升高和双侧颞叶实质内病变的颅磁共振成像证据,提示边缘脑炎。在排除其他诊断后,要求筛查CSF中的自身抗体,结果抗lgi1抗体阳性。假设诊断为抗lgi1 ALE,并在临床和影像学显著改善的情况下开始治疗。结论:ALE的广泛临床谱导致诊断不足。因此,对于新发神经精神症状且无精神病史的患者,应考虑ALE,及时诊断和治疗是获得良好预后的关键。学习要点:自身免疫性边缘脑炎是一种罕见的炎症性神经系统疾病,影响边缘系统,特别是海马体,导致记忆障碍和神经精神症状。由于其广泛的神经精神症状,自身免疫性边缘脑炎的诊断可能被忽视,导致误诊为另一种医学疾病。早期诊断对于通过适当治疗预防潜在的神经系统后遗症至关重要。
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来源期刊
CiteScore
2.10
自引率
0.00%
发文量
166
审稿时长
8 weeks
期刊介绍: The European Journal of Case Reports in Internal Medicine is an official journal of the European Federation of Internal Medicine (EFIM), representing 35 national societies from 33 European countries. The Journal''s mission is to promote the best medical practice and innovation in the field of acute and general medicine. It also provides a forum for internal medicine doctors where they can share new approaches with the aim of improving diagnostic and clinical skills in this field. EJCRIM welcomes high-quality case reports describing unusual or complex cases that an internist may encounter in everyday practice. The cases should either demonstrate the appropriateness of a diagnostic/therapeutic approach, describe a new procedure or maneuver, or show unusual manifestations of a disease or unexpected reactions. The Journal only accepts and publishes those case reports whose learning points provide new insight and/or contribute to advancing medical knowledge both in terms of diagnostics and therapeutic approaches. Case reports of medical errors, therefore, are also welcome as long as they provide innovative measures on how to prevent them in the current practice (Instructive Errors). The Journal may also consider brief and reasoned reports on issues relevant to the practice of Internal Medicine, as well as Abstracts submitted to the scientific meetings of acknowledged medical societies.
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