Abstract
This retrospective study aimed to investigate the clinical features associated with deteriorated swallow in amyotrophic lateral sclerosis (ALS) patients with spinal and bulbar onset, describe the modification of diet and liquid intake, and assess the impact of dysphagia on the use of riluzole. One hundred forty-five patients were observed periodically every 3–6 months. They underwent routinely fiberoptic endoscopic evaluation of swallowing (FEES) and spirometry; dysphagia severity was classified according to the Penetration Aspiration Scale and the Pooling score (P-score) integrated with other parameters such as sensation, collaboration, and age (P-SCA score). During a mean follow-up period of about 2 years, the percentage of ALS patients suffering from dysphagia increased to 85 (rising from 35 to 73% in patients with spinal onset and from 95 to 98% in those with bulbar onset). Also, 8% of patients with dysphagia by FEES did not perceive the disorder. The frequency of normal and semi-solid diets decreased over time, while that of pureed diets and percutaneous endoscopic gastrostomy (PEG) prescription increased. Forty-four percent of dysphagic patients refused thickeners or PEG. A significant difference was observed in the mortality rate between patients untreated with riluzole and patients treated with riluzole oral suspension (p < 0.05). Disease duration mainly impacted on the frequency of dysphagia in spinal onset patients, appearing very early in those with bulbar onset. Riluzole oral suspension would allow the safe administration in dysphagic ALS patients to avoid tablet crushing and consequent dispersion in food, common practices that are inconsistent with the safe and effective use of the drug.
Highlights
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by a progressive loss of motor neurons that leads to paralysis and death within 2–5 years from the time of diagnosis [1]
We aimed to investigate the impact of dysphagia on diet and riluzole intake, analyze the risk of dysphagia in patients unable to recognize disease symptoms, and evaluate the effect of riluzole taken in an uncommon way on survival
We evaluated the clinical features associated with dysphagia in ALS patients and their impact on riluzole and food management
Summary
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by a progressive loss of motor neurons that leads to paralysis and death within 2–5 years from the time of diagnosis [1]. Death occurs because of respiratory failure, aspiration pneumonia, malnutrition, and dehydration [2]. About one-third of ALS patients show a bulbar onset with dysphagia and dysarthria. Independent of the clinical onset, dysphagia emerges in more than 80% of patients during the advanced. A prompt assessment of the swallowing function is crucial to organize proper interventions and prevent rapid clinical deterioration [5]. The endoscopic assessment allows a direct examination of the laryngeal adductor reflex (LAR) and can be repeated as often as necessary due to absence of radiation exposure
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