Hillel A D, Miller R
Department of Otolaryngology/Head & Neck Surgery, University of Washington, Seattle.
Head Neck. 1989 Jan-Feb;11(1):51-9. doi: 10.1002/hed.2880110110.
Patients with bulbar amyotrophic lateral sclerosis (ALS) are often referred to the otolaryngologist/head and neck surgeon and speech pathologist for evaluation and management of dysphagia and dysarthria. These patients comprise an unusual group because of the progressive and multi-system nature of their illness. The neuromuscular disabilities associated with bulbar ALS cause a myriad of related symptoms associated with swallowing, speech, and respiration. Although the rate of progression cannot be predicted, a general pattern of progression is noted. Bulbar disease accounts for the majority of the worst symptoms of ALS. The loss of the ability to swallow changes eating from a pleasurable task to a burden of survival. Loss of communication effectively imprisons the patient in a state of isolation. The progressive weakness of respiration, predominantly a spinal rather than bulbar manifestation, is the cause of death for nearly all ALS patients and is also discussed. The general patterns of progression of bulbar ALS are outlined in this paper. The development of symptoms are correlated with specific treatment recommendations to aid the clinician in devising an orderly plan of management for this progressive disease.
延髓性肌萎缩侧索硬化症（ALS）患者常被转诊至耳鼻喉科医生/头颈外科医生及言语病理学家处，以评估和处理吞咽困难及构音障碍。由于其病情呈进行性且累及多系统，这些患者构成了一个特殊群体。与延髓性ALS相关的神经肌肉残疾会引发一系列与吞咽、言语和呼吸相关的症状。尽管疾病进展速度无法预测，但仍可观察到一种大致的进展模式。延髓性疾病是ALS最严重症状的主要原因。吞咽能力丧失使进食从一项愉悦的活动变成了生存负担。沟通能力丧失实际上将患者禁锢在孤立状态。呼吸功能进行性减弱主要是脊髓而非延髓的表现，几乎是所有ALS患者的死因，本文也对此进行了讨论。本文概述了延髓性ALS的大致进展模式。症状的发展与具体治疗建议相关联，以帮助临床医生为这种进行性疾病制定有序的管理计划。
