Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 Mar 21:8:94.
doi: 10.3389/fneur.2017.00094. eCollection 2017.

Dysphagia in Amyotrophic Lateral Sclerosis: Impact on Patient Behavior, Diet Adaptation, and Riluzole Management

Emanuela Onesti  1 , Ilenia Schettino  2 , Maria Cristina Gori  1 , Vittorio Frasca  1 , Marco Ceccanti  1 , Chiara Cambieri  1 , Giovanni Ruoppolo  2 , Maurizio Inghilleri  1
Affiliations

Dysphagia in Amyotrophic Lateral Sclerosis: Impact on Patient Behavior, Diet Adaptation, and Riluzole Management

Emanuela Onesti et al. Front Neurol. .

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.

Keywords: amyotrophic lateral sclerosis; diet; dysphagia; riluzole; swallow.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Incidence of dysphagia in the whole amyotrophic lateral sclerosis population and according to spinal or bulbar onset at the beginning and the end of follow-up.
Figure 2
Figure 2
Change in Penetration Aspiration Scale (PAS) score during the follow-up period in spinal and bulbar patients. Current effect: F(2.284) = 15.210, p = 0.001. Vertical bars denote 0.95 confidence intervals.
Figure 3
Figure 3
The fiberoptic endoscopic evaluation of swallowing of one amyotrophic lateral sclerosis patient who did not perceive the objectified disorder but with abundant hypopharyngeal residues.
Figure 4
Figure 4
Administration of riluzole in amyotrophic lateral sclerosis (ALS) patients according to dysphagia.
See this image and copyright information in PMC

References

    1. Kiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, et al. Amyotrophic lateral sclerosis. Lancet (2011) 377(9769):942–55.10.1016/S0140-6736(10)61156-7 - DOI - PubMed
    1. Czaplinski A, Yen AA, Simpson EP, Appel SH. Predictability of disease progression in amyotrophic lateral sclerosis. Muscle Nerve (2006) 34:702–8.10.1002/mus.20658 - DOI - PubMed
    1. Muscaritoli M, Kushta I, Molfino A, Inghilleri M, Sabatelli M, Rossi Fanelli F. Nutritional and metabolic support in patients with amyotrophic lateral sclerosis. Nutrition (2012) 28(10):959–66.10.1016/j.nut.2012.01.011 - DOI - PubMed
    1. Luchesi KF, Kitamura S, Mourão LF. Management of dysphagia in Parkinson’s disease and amyotrophic lateral sclerosis. Codas (2013) 25(4):358–64.10.1590/S2317-17822013000400010 - DOI - PubMed
    1. Kühnlein P, Gdynia HJ, Sperfeld AD, Lindner-Pfleghar B, Ludolph AC, Prosiegel M, et al. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Clin Pract Neurol (2008) 4:366–74.10.1038/ncpneuro0853 - DOI - PubMed

LinkOut - more resources