Laryngospasm in amyotrophic lateral sclerosis

Abstract

Introduction: Laryngospasm is an involuntary, sustained closure of sphincter musculature that leads to an unpleasant subjective experience of dyspnea and choking. It is an underreported symptom in amyotrophic lateral sclerosis (ALS). In this study we aimed to better characterize the prevalence and clinical characteristics of laryngospasm in ALS patients. Methods:The medical records of 571 patients with ALS followed between 2008 and 2018 were searched for evidence of laryngospasm. A total of 23 patients with laryngospasm were identified and the data related to patient and laryngospasm characteristics were extracted.Results: Laryngospasm was reported in 4% of ALS patients. Females comprised 57% of patients and their mean age was 63.4 years. Laryngospasm frequently manifested in patients with moderate bulbar dysfunction and seemed independent of respiratory function. Among laryngospasm patients, 26% were cigarette smokers and 13% had a history of gastroesophageal reflux. The most common reported trigger was excessive saliva irritating the vocal cords (35%) followed by eating a meal (17%). There was significant variation in laryngospasm frequency (up to 5 per hour) and duration (seconds to minutes). Most patients could not identify an effective coping mechanism, although 13% reported that drinking water was effective.Discussion: Despite its low prevalence in ALS, laryngospasm should be included in the symptom inquiry. The present findings may improve patient care through increased recognition of the clinical features of laryngospasm in ALS patients, identifying a link between laryngospasm and moderate bulbar dysfunction, and highlighting trigger avoidance as a management strategy. Additional research is required to understand the pathophysiology and optimal treatment.

Laryngospasm: A frequently underrecognized symptom in amyotrophic lateral sclerosis

“…Contrary to prior data on laryngospasm in patients with SBMA 6 or other neurologic conditions, 2 the authors found that smoking and gastroesophageal reflux disease (GERD) were not major risk factors in patients with ALS. Instead, aspirating secretions was the most common trigger in the present study, reported in more than a third of patients, a frequency more than double that of the next most common trigger 1 . This suggests that sialorrhea, or more precisely pseudo‐sialorrhea due to bulbar dysfunction, is one major risk factor predisposing to vocal cord irritation and laryngospasm in patients with ALS.…”

“…The authors report that their patients' most commonly used therapies for laryngospasm were relatively ineffective, with most showing benefit in less than 10% of patients. 1 The lack of effective treatments, as perceived by those affected, may indicate lack of understanding on the part of patients about the triggers discussed above, and consequently uncertainty of the cause of their laryngospasm and how to prevent or mitigate it. This highlights an important need for education and for shared decision-making about symptom management, especially because laryngospasms were occasionally frequent and severe.…”

“…Instead, aspirating secretions was the most common trigger in the present study, reported in more than a third of patients, a frequency more than double that of the next most common trigger. 1 This suggests that sialorrhea, or more precisely pseudo-sialorrhea due to bulbar dysfunction, is one major risk factor predisposing to vocal cord irritation and laryngospasm in patients with ALS. Consequently, treatments that reduce saliva production or improve its management, such as anticholinergic agents, intermittent suction, or botulinum toxin injections into the salivary glands, may prove effective.…”

“…In this issue of Muscle & Nerve , Gotesman et al report their findings from a retrospective review of laryngospasm in a large single‐center cohort of patients with amyotrophic lateral sclerosis (ALS) 1 . Laryngospasm is defined as a paroxysmal episode beginning with the subjective sensation of inability to move air, followed by inspiratory stridor associated with rapid and forceful contraction of the laryngeal sphincter 2 .…”

Laryngospasm Causing Recurrent Negative Pressure Alveolar Hemorrhage Resembling Acute Exacerbation of Interstitial Lung Disease: A Case Report

“…Laryngospasm is distinct from induced laryngeal obstruction (ILO), a recently proposed disease concept that causes relatively mild breathing problems mainly on exertion 8) . The causes of laryngospasm may include endotracheal maneuvers, reflux esophagitis, amyotrophic lateral sclerosis, and hypomagnesemia [9][10][11] . Idiopathic cases of recurrent laryngospasm with no apparent cause have also been reported 12,13) .…”