[Analysis of clinical characteristics of amyotrophic lateral sclerosis patients initially diagnosed with abnormal laryngeal function]

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2024 Dec 19:59:1293-1298. doi: 10.3760/cma.j.cn115330-20240630-00388. Online ahead of print.
[Article in Chinese]

Abstract

Objective: To study the laryngeal functional characteristics of patients with amyotrophic lateral sclerosis (ALS)disease diagnosed at the voice clinic. Methods: A retrospective analysis(case series study) was conducted on the laryngeal functional characteristics of 7 patients [2 males, 5 females, age ranged from 43 to 76(60.85±13.18)]with motor neuron disease who visited the voice clinic and were ultimately diagnosed by neurologists. The data included laryngostroboscopy, fiberoptic endoscopic examination of swallowing(FEES), acoustic analysis and laryngeal electromyography(LEMG). Descriptive methods were used for analysis. Results: ①There were 2 males and 5 females, with an average age of (60.85±13.18) years. They had previously visited the otolaryngology department more than twice, visit frequency with an average of 3.57 and an average diagnosis time of 12.28 months. The main complaints of the patient at the time of treatment were voice change, dysphagia or vocal fatigue. ②LEMG: Among 7 cases, 4 cases demonstrated neurogenic damage, all of which were bilateral, and 3 cases showed normal findings on examination. Spontaneous potentials (SP) were present in three cases for more than 6 months, with the longest duration being 24 months. Three cases exhibited the coexistence of spontaneous potential and reinnervated motor unit potentials (MUPs), and two cases showed bundle tremor potential.③Laryngostroboscopy revealed bilateral vocal fold asymmetry and glottic insufficiency in 7 cases, and decreased vocal cord movement in 4 cases, and vocal cord atrophy in 5 cases. FEES showed that 7 patients presented with mild to severe swallowing dysfunction, 3 cases had soft palate insufficiency and mild to severe food residues in the epiglottic valley and pyriform fossa. 1 case showed leakage and 1 case showed aspiration. Conclusions: Patients presenting with initial symptoms of abnormal laryngeal function should be vigilant for the possibility of motor neuron disease, especially when laryngostroboscopy reveals abnormal vocal fold movement and swallowing dysfunction. LEMG examination reveals bilateral neurogenic damage, prolonged spontaneous potential, coexistence of spontaneous potential and reinnervated MUPs, and the appearance of bundle tremor potential, which is beneficial for early detection of motor neuron disease.

目的: 分析首诊嗓音科的肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)患者的喉部症状、体征和喉肌电图特点。 方法: 该病例系列研究分析2021年4月至2023年4月在厦门大学附属中山医院嗓音门诊首诊、最终确诊ALS的7例患者[男性2例,女性5例;年龄为43~76(60.85±13.18)岁]的喉部症状、体征(频闪喉镜、吞咽喉镜)、声学评估及喉肌电图资料。采用描述性方法进行分析。 结果: ①7例患者既往均在耳鼻咽喉科就诊2次以上(中位次数3.57次),确诊时间为12.28个月。就诊时主诉主要为:声音嘶哑、发声费力、发声疲劳、吞咽异物感或吞咽困难、呼吸不畅及说话含糊等。②喉肌电图:7例中4例提示为神经源性损害,且均为双侧,3例检查正常;3例发现自发电位者均出现在6个月以上,最长者达24个月;3例发现进行性失神经损害和慢性再生并存,2例出现束颤电位。③频闪喉镜:7例患者均出现双侧声带运动不对称和声门闭合不全(4例垂直面,3例水平面),5例声带松弛,3例双侧声带运动减弱,1例单侧声带运动减弱。吞咽喉镜:7例患者均显示有轻-重度不等的吞咽功能障碍,3例患者软腭闭合不全,进食后会厌谷及梨状窝均有轻度-重度不等的食物残留,1例见渗漏,1例见误吸。 结论: 首发症状为喉功能异常的患者,当频闪喉镜发现声带运动功能异常特别是声门闭合不全同时伴有吞咽喉镜下吞咽功能异常时,需警惕ALS的可能,喉肌电图检查发现双侧神经源性损害、自发电位长时间持续存在、自发电位和宽大运动单位电位(motor unit potential,MUP)并存以及束颤电位的出现,有助于早期发现诊断ALS。.

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