{"title":"抗 LGI1 自身免疫性边缘脑炎:容易被忽视的诊断","authors":"Carolina Freitas Henriques, Rui Fernandes, Leonor Neves, Rubina Miranda, Duarte Noronha, Teresa Carolina Aguiar, Teresa Faria","doi":"10.12890/2024_005025","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Case description: </strong>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.</p><p><strong>Conclusions: </strong>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.</p><p><strong>Learning points: </strong>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.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"005025"},"PeriodicalIF":0.0000,"publicationDate":"2024-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11623357/pdf/","citationCount":"0","resultStr":"{\"title\":\"Anti-LGI1 Autoimmune Limbic Encephalitis: An Easy-To-Miss Diagnosis.\",\"authors\":\"Carolina Freitas Henriques, Rui Fernandes, Leonor Neves, Rubina Miranda, Duarte Noronha, Teresa Carolina Aguiar, Teresa Faria\",\"doi\":\"10.12890/2024_005025\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>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.</p><p><strong>Case description: </strong>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.</p><p><strong>Conclusions: </strong>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.</p><p><strong>Learning points: </strong>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.</p>\",\"PeriodicalId\":11908,\"journal\":{\"name\":\"European journal of case reports in internal medicine\",\"volume\":\"11 12\",\"pages\":\"005025\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-11-19\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11623357/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"European journal of case reports in internal medicine\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.12890/2024_005025\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"European journal of case reports in internal medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.12890/2024_005025","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
Anti-LGI1 Autoimmune Limbic Encephalitis: An Easy-To-Miss Diagnosis.
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.
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.