Upper airway manifestations of anti-IgLON5 disease: Otorhinolaryngological point of view

IF 0.9 Q3 OTORHINOLARYNGOLOGY Acta otorrinolaringologica espanola Pub Date : 2024-09-01 DOI:10.1016/j.otorri.2024.04.001
Daniela Pastene , Eduardo Lehrer , Sara Jubes , Joan Santamaria , Alex Iranzo , Carles Gaig , Isabel Vilaseca
{"title":"Upper airway manifestations of anti-IgLON5 disease: Otorhinolaryngological point of view","authors":"Daniela Pastene ,&nbsp;Eduardo Lehrer ,&nbsp;Sara Jubes ,&nbsp;Joan Santamaria ,&nbsp;Alex Iranzo ,&nbsp;Carles Gaig ,&nbsp;Isabel Vilaseca","doi":"10.1016/j.otorri.2024.04.001","DOIUrl":null,"url":null,"abstract":"<div><h3>Introduction</h3><p>Anti-IgLON5 disease is a recently described neurological disorder with multisystemic features. The disease is characterized by the presence of IgLON5 antibodies in serum and cerebrospinal fluid. Our objective is to describe in detail the otorhinolaryngological manifestations of this disease, which are frequent and may include dysphagia, dysarthria, vocal cord paralysis and laryngospasm.</p></div><div><h3>Methods</h3><p>In this study, we present a series of 9 patients with anti-IgLON5 disease and otolaryngological manifestations. Patients were evaluated between July 2012 and March 2022 by video-polysomnography, fiber-optic laryngoscopy, and functional endoscopic evaluation of swallowing.</p></div><div><h3>Results</h3><p>The median age was 71 years, and 5 (56%) were female. Video-polysomnography showed a NREM/REM parasomnia in 6 patients (67%), obstructive sleep apnea in 8 (88%), stridor during sleep in 7 (78%) and central apneas in 1 (11%). Six out of the 9 patients (67%) presented episodes of acute respiratory failure that required mechanical ventilation, 6 had vocal fold palsy with 4 of them requiring tracheostomy (3 had to be performed on an emergency basis). Dysphagia occurred in 8 patients (89%). Prominent upper airway secretion and sialorrhea was also present in 3 cases.</p></div><div><h3>Conclusion</h3><p>The anti-IgLON5 disease exhibits extensive otolaryngological symptoms, mainly affecting the upper airway. These symptoms affect the quality of life and can be life-threatening. Prompt acute management is essential for stridor, dyspnea, and dysphagia. Given the potential severity of the symptoms and rarity of the disease, it is important for otolaryngologists to be familiar with anti-IgLON5 disease.</p></div><div><h3>Level of evidence</h3><p>Level 4.</p></div>","PeriodicalId":7019,"journal":{"name":"Acta otorrinolaringologica espanola","volume":"75 5","pages":"Pages 288-295"},"PeriodicalIF":0.9000,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta otorrinolaringologica espanola","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0001651924000505","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"OTORHINOLARYNGOLOGY","Score":null,"Total":0}
引用次数: 0

Abstract

Introduction

Anti-IgLON5 disease is a recently described neurological disorder with multisystemic features. The disease is characterized by the presence of IgLON5 antibodies in serum and cerebrospinal fluid. Our objective is to describe in detail the otorhinolaryngological manifestations of this disease, which are frequent and may include dysphagia, dysarthria, vocal cord paralysis and laryngospasm.

Methods

In this study, we present a series of 9 patients with anti-IgLON5 disease and otolaryngological manifestations. Patients were evaluated between July 2012 and March 2022 by video-polysomnography, fiber-optic laryngoscopy, and functional endoscopic evaluation of swallowing.

Results

The median age was 71 years, and 5 (56%) were female. Video-polysomnography showed a NREM/REM parasomnia in 6 patients (67%), obstructive sleep apnea in 8 (88%), stridor during sleep in 7 (78%) and central apneas in 1 (11%). Six out of the 9 patients (67%) presented episodes of acute respiratory failure that required mechanical ventilation, 6 had vocal fold palsy with 4 of them requiring tracheostomy (3 had to be performed on an emergency basis). Dysphagia occurred in 8 patients (89%). Prominent upper airway secretion and sialorrhea was also present in 3 cases.

Conclusion

The anti-IgLON5 disease exhibits extensive otolaryngological symptoms, mainly affecting the upper airway. These symptoms affect the quality of life and can be life-threatening. Prompt acute management is essential for stridor, dyspnea, and dysphagia. Given the potential severity of the symptoms and rarity of the disease, it is important for otolaryngologists to be familiar with anti-IgLON5 disease.

Level of evidence

Level 4.

查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
抗IgLON5疾病的上呼吸道表现:耳鼻喉科观点
导言抗 IgLON5 病是最近描述的一种具有多系统特征的神经系统疾病。该病的特征是血清和脑脊液中存在 IgLON5 抗体。我们的目的是详细描述该病的耳鼻喉科表现,这些表现很常见,可能包括吞咽困难、构音障碍、声带麻痹和喉痉挛。2012年7月至2022年3月期间,我们通过视频多导睡眠图、纤维喉镜和吞咽功能内窥镜对患者进行了评估。视频多导睡眠图显示,6 名患者(67%)患有 NREM/REM寄生虫性失眠,8 名患者(88%)患有阻塞性睡眠呼吸暂停,7 名患者(78%)在睡眠中出现呼吸困难,1 名患者(11%)出现中枢性呼吸暂停。9 名患者中有 6 人(67%)出现急性呼吸衰竭,需要进行机械通气,6 人出现声带麻痹,其中 4 人需要进行气管造口术(3 人必须紧急进行)。8名患者(89%)出现吞咽困难。结论抗 IgLON5 疾病表现出广泛的耳鼻喉症状,主要影响上呼吸道。这些症状会影响患者的生活质量,甚至危及生命。对于喘鸣、呼吸困难和吞咽困难,及时的急性处理至关重要。鉴于症状的潜在严重性和疾病的罕见性,耳鼻喉科医生必须熟悉抗IgLON5疾病。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 去求助
来源期刊
CiteScore
1.40
自引率
20.00%
发文量
44
审稿时长
44 days
期刊介绍: Es la revista más importante en español dedicada a la especialidad. Ofrece progresos científicos y técnicos tanto a nivel de originales como de casos clínicos. Además, es la Publicación Oficial de la Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial y está presente en los más prestigiosos índices de referencia.
期刊最新文献
Retrolabyrinthine approach to the lateral skull base: The value of preoperative temporal bone CT analysis Situación actual de la tutoría en la formación especializada en otorrinolaringología en España. ¿Dónde estamos? ¿Hacia dónde queremos ir? Prevalencia de apnea obstructiva del sueño en pacientes obesos candidatos a cirugía bariátrica y cuestionarios predictores Recurrent peritonsillar abscess in adults: Incidence and risk factors in a prospective longitudinal cohort Enhancing the coverage and rotation of anterior ethmoidal artery flap for septal perforation closure: Insights from a flap design study
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1