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Improvement in mild anti-IgLON5 encephalopathy without immunotherapy: a case report

BACKGROUND: Anti-IgLON5 antibody-related encephalopathy is a recently discovered and rare autoimmune disease, and its diagnosis and treatment are more challenging than for other autoimmune encephalopathic diseases. Sleep disorder is the most prominent symptom of the disease. It can also present with...

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Autores principales: Wang, Yuting, Wu, Xiuling, Lu, Baoquan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7968182/
https://www.ncbi.nlm.nih.gov/pubmed/33731000
http://dx.doi.org/10.1186/s12883-021-02145-4
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author Wang, Yuting
Wu, Xiuling
Lu, Baoquan
author_facet Wang, Yuting
Wu, Xiuling
Lu, Baoquan
author_sort Wang, Yuting
collection PubMed
description BACKGROUND: Anti-IgLON5 antibody-related encephalopathy is a recently discovered and rare autoimmune disease, and its diagnosis and treatment are more challenging than for other autoimmune encephalopathic diseases. Sleep disorder is the most prominent symptom of the disease. It can also present with gait instability, dysarthria, dysphagia, dementia, ataxia, autonomic nervous system dysfunction, chorea, vertical gaze paralysis, and other symptoms. Immunotherapy remains the primary treatment for this disease; however, there is no definitive conclusion regarding the effect of immunotherapy. The clinical symptoms of the reported cases of anti-IgLON5 antibody-related encephalopathy were generally severe. However, the symptoms in our patient were mild and relieved without immunotherapy, unlike the previously reported cases. CASE PRESENTATION: A 62-year-old man presented with behavioural abnormalities and involuntary movements after nearly 2 months of fever and headache. He also had symptoms of mild sleep disorder. Due to the abnormal levels of infection-related indicators, antiviral treatment was started on the day of admission. The serum analysis confirmed the presence of IgLON5 antibody, and the patient was found to be genetically susceptible. The patient’s symptoms resolved rapidly without immunotherapy and did not recur. CONCLUSIONS: This case demonstrated that IgLON5 antibody-related encephalopathy might have mild manifestations. Infection and a genetic predisposition may be important causes for the disease. Patients with a mild disease may have a better prognosis.
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spelling pubmed-79681822021-03-22 Improvement in mild anti-IgLON5 encephalopathy without immunotherapy: a case report Wang, Yuting Wu, Xiuling Lu, Baoquan BMC Neurol Case Report BACKGROUND: Anti-IgLON5 antibody-related encephalopathy is a recently discovered and rare autoimmune disease, and its diagnosis and treatment are more challenging than for other autoimmune encephalopathic diseases. Sleep disorder is the most prominent symptom of the disease. It can also present with gait instability, dysarthria, dysphagia, dementia, ataxia, autonomic nervous system dysfunction, chorea, vertical gaze paralysis, and other symptoms. Immunotherapy remains the primary treatment for this disease; however, there is no definitive conclusion regarding the effect of immunotherapy. The clinical symptoms of the reported cases of anti-IgLON5 antibody-related encephalopathy were generally severe. However, the symptoms in our patient were mild and relieved without immunotherapy, unlike the previously reported cases. CASE PRESENTATION: A 62-year-old man presented with behavioural abnormalities and involuntary movements after nearly 2 months of fever and headache. He also had symptoms of mild sleep disorder. Due to the abnormal levels of infection-related indicators, antiviral treatment was started on the day of admission. The serum analysis confirmed the presence of IgLON5 antibody, and the patient was found to be genetically susceptible. The patient’s symptoms resolved rapidly without immunotherapy and did not recur. CONCLUSIONS: This case demonstrated that IgLON5 antibody-related encephalopathy might have mild manifestations. Infection and a genetic predisposition may be important causes for the disease. Patients with a mild disease may have a better prognosis. BioMed Central 2021-03-17 /pmc/articles/PMC7968182/ /pubmed/33731000 http://dx.doi.org/10.1186/s12883-021-02145-4 Text en © The Author(s) 2021 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Wang, Yuting
Wu, Xiuling
Lu, Baoquan
Improvement in mild anti-IgLON5 encephalopathy without immunotherapy: a case report
title Improvement in mild anti-IgLON5 encephalopathy without immunotherapy: a case report
title_full Improvement in mild anti-IgLON5 encephalopathy without immunotherapy: a case report
title_fullStr Improvement in mild anti-IgLON5 encephalopathy without immunotherapy: a case report
title_full_unstemmed Improvement in mild anti-IgLON5 encephalopathy without immunotherapy: a case report
title_short Improvement in mild anti-IgLON5 encephalopathy without immunotherapy: a case report
title_sort improvement in mild anti-iglon5 encephalopathy without immunotherapy: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7968182/
https://www.ncbi.nlm.nih.gov/pubmed/33731000
http://dx.doi.org/10.1186/s12883-021-02145-4
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