Otorhinolaryngology Department, Hospital Clínic, Barcelona, Spain.
Otorhinolaryngology Department, Hospital Clínic, Barcelona, Spain; Head Neck Cancer Unit, Hospital Clínic, Barcelona, Spain; Department of Surgery and Medical Surgical Specialties, School of Medicine, Universitat de Barcelona, Spain.
Acta Otorrinolaringol Esp (Engl Ed). 2024 Sep-Oct;75(5):288-295. doi: 10.1016/j.otoeng.2024.04.001. Epub 2024 May 8.
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.
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.
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.
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 4.
抗 IgLON5 病是一种最近描述的具有多系统特征的神经疾病。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。
在这项研究中，我们介绍了 9 例具有耳鼻喉科表现的抗 IgLON5 病患者。患者于 2012 年 7 月至 2022 年 3 月通过视频多导睡眠图、纤维喉镜和吞咽功能内镜评估进行评估。
中位年龄为 71 岁，5 例（56%）为女性。6 例（67%）患者的视频多导睡眠图显示非快速眼动/快速眼动睡眠行为障碍，8 例（88%）存在阻塞性睡眠呼吸暂停，7 例（78%）在睡眠期间出现喘鸣，1 例（11%）出现中枢性呼吸暂停。9 例患者中有 6 例（67%）出现急性呼吸衰竭发作，需要机械通气，6 例存在声带麻痹，其中 4 例需要气管切开术（3 例需要紧急进行）。8 例（89%）患者存在吞咽困难。3 例还存在明显的上气道分泌物和流涎。
抗 IgLON5 病表现出广泛的耳鼻喉科症状，主要影响上呼吸道。这些症状会影响生活质量，并可能危及生命。对于喘鸣、呼吸困难和吞咽困难，及时进行急性处理至关重要。鉴于症状的潜在严重程度和疾病的罕见性，耳鼻喉科医生熟悉抗 IgLON5 病非常重要。
4 级。
