Vocal Cord Dysfunction in Amyotrophic Lateral Sclerosis

Abstract

e describe 4 patients with amyotrophic lateral sclerosis (ALS) and glottic narrowing due to vocal cord dysfunction, and review the literature found using the following search terms: amyotrophic lateral sclerosis, motor neuron disease, stridor, laryngospasm, vocal cord abductor paresis, and hoarseness. Neurological literature rarely reports vocal cord dysfunction in ALS, in contrast to otolaryngology literature (4%-30% of patients with ALS). Both infranuclear and supranuclear mechanisms may play a role. Vocal cord dysfunction can occur at any stage of disease and may account for sudden death in ALS. Treatment of severe cases includes acute airway management and tracheotomy.

Laryngospasm as preceding symptom of amyotrophic lateral sclerosis

“…In a large cohort of 44 ALS patients with initial bulbar symptoms, none had isolated laryngospasm [5]. Laryngospasm is reported in 2% of ALS patients in early clinical stage, but seems to be more frequent in later stages of the disease [6,7].…”

Reduced survival in patients with ALS with upper airway obstructive events on non-invasive ventilation

“…We did not have enough data to differentiate between bulbar and pseudobulbar patients, a differentiation that should be explored in future studies. Finally, other mechanisms, described in different diseases, may also be responsible for upper airway obstruction; these include, for example, chronic irritation of the surface of the pharynx,27 28 a higher propensity of the airways to collapse at low lung volume29 30 or upper airway nocturnal redistribution of interstitial fluid 31…”

The Impact of Aging and Progressive Neurological Disease on Swallowing: A Concise Overview

“…The dysphagia will occur in relation to progressive weakness (due to flaccidity and spasticity) of oropharyngeal musculature. Laryngospasms may occur for some (van der Graaff et al 2009). Malnutrition may lead to further progression of muscle weakness (Worwood and Leigh 1998;Heffernan et al 2004).…”