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Four Cases and a Review of the Literature

Maaike M. van der Graaff, MD; Wilko Grolman, MD, PhD; Erik J. Westermann, MD; Hans C. Boogaardt; Hans Koelman, MD, PhD; Anneke J. van der Kooi, MD, PhD; Marina A. Tijssen, MD, PhD; Marianne de Visser, MD, PhD

Arch Neurol. 2009;66(11):1329-1333.

We 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.

Author Affiliations: Departments of Neurology (Drs van der Graaff, van der Kooi, Tijssen, and de Visser), Otolaryngology (Dr Grolman and Mr Boogaardt), and Neurophysiology (Dr Koelman), Academic Medical Center, Amsterdam; and Center for Home Mechanical Ventilation (Dr Westermann), University Medical Center Utrecht, Utrecht, the Netherlands.

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