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Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis

Abstract

Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease of the motor system. Bulbar symptoms such as dysphagia and dysarthria are frequent features of ALS and can result in reductions in life expectancy and quality of life. These dysfunctions are assessed by clinical examination and by use of instrumented methods such as fiberendoscopic evaluation of swallowing and videofluoroscopy. Laryngospasm, another well-known complication of ALS, commonly comes to light during intubation and extubation procedures in patients undergoing surgery. Laryngeal and pharyngeal complications are treated by use of an array of measures, including body positioning, compensatory techniques, voice and breathing exercises, communication devices, dietary modifications, various safety strategies, and neuropsychological assistance. Meticulous monitoring of clinical symptoms and close cooperation within a multidisciplinary team (physicians, speech and language therapists, occupational therapists, dietitians, caregivers, the patients and their relatives) are vital.

Key Points

  • Bulbar symptoms such as dysarthria and dysphagia are frequent features of amyotrophic lateral sclerosis (ALS) and can reduce life expectancy and quality of life

  • Dysarthria results from flaccid or spastic paresis of the musculature of the face, tongue, lips, palate, pharynx and larynx

  • Dysphagia can result from weakness or spasticity of the muscles innervated by trigeminal, facial, hypoglossal, glossopharyngeal or vagal nerves

  • Laryngospasm, which is defined as a paroxysmal episode with the sensation that air cannot be moved in and out, accompanied by inspiratory stridor, affects up to 19% of patients with ALS

  • Speech therapy can be helpful in the early stages of ALS to correct ineffective compensation strategies; augmentative and alternative communication devices should be provided as the disease progresses

  • Compensatory and restitutional methods and dietary modifications should be used to ensure oral alimentation for as long as possible and to prevent aspiration of food; enteral nutrition should be considered as dysphagia increases

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Acknowledgements

The authors thank Margit Friedrich and Falk Schradt for helpful comments and Katarina Groth for proofreading of the manuscript. Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Correspondence to Axel Riecker.

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Kühnlein, P., Gdynia, HJ., Sperfeld, AD. et al. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Rev Neurol 4, 366–374 (2008). https://doi.org/10.1038/ncpneuro0853

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