Abstract：
BACKGROUND: 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.
CONCLUSIONS: 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.
METHODS: Level 4.
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.
CONCLUSIONS: 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.
METHODS: Level 4.
摘要：
背景：抗IgLON5疾病是一种最近描述的具有多系统特征的神经系统疾病。该疾病的特征在于血清和脑脊液中存在IgLON5抗体。我们的目的是详细描述这种疾病的耳鼻咽喉科表现，经常发生，可能包括吞咽困难，构音障碍,声带麻痹和喉痉挛。
方法：在本研究中，我们介绍了9例抗IgLON5疾病和耳鼻喉科表现的患者。在2012年7月至2022年3月期间，通过视频多导睡眠图对患者进行了评估，光纤喉镜,吞咽功能的内镜评价。
结果：中位年龄为71岁，5名(56%)为女性。视频多导睡眠图显示6例患者（67%）出现NREM/REM失眠症，8例（88%）阻塞性睡眠呼吸暂停，睡眠时喘鸣7例（78％），中枢呼吸暂停1例（11％）。9例患者中有6例（67%）出现需要机械通气的急性呼吸衰竭。6例患有声带麻痹，其中4例需要气管造口术（3例必须在紧急情况下进行）。吞咽困难8例(89%)。3例患者还出现明显的上呼吸道分泌和流涕。
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。这些症状影响生活质量，可能危及生命。及时的急性治疗对于喘鸣至关重要，呼吸困难,和吞咽困难.鉴于症状的潜在严重程度和疾病的罕见性，耳鼻喉科医师熟悉抗IgLON5疾病很重要。
方法：第4级。
方法：在本研究中，我们介绍了9例抗IgLON5疾病和耳鼻喉科表现的患者。在2012年7月至2022年3月期间，通过视频多导睡眠图对患者进行了评估，光纤喉镜,吞咽功能的内镜评价。
结果：中位年龄为71岁，5名(56%)为女性。视频多导睡眠图显示6例患者（67%）出现NREM/REM失眠症，8例（88%）阻塞性睡眠呼吸暂停，睡眠时喘鸣7例（78％），中枢呼吸暂停1例（11％）。9例患者中有6例（67%）出现需要机械通气的急性呼吸衰竭。6例患有声带麻痹，其中4例需要气管造口术（3例必须在紧急情况下进行）。吞咽困难8例(89%)。3例患者还出现明显的上呼吸道分泌和流涕。
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。这些症状影响生活质量，可能危及生命。及时的急性治疗对于喘鸣至关重要，呼吸困难,和吞咽困难.鉴于症状的潜在严重程度和疾病的罕见性，耳鼻喉科医师熟悉抗IgLON5疾病很重要。
方法：第4级。
