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 Access through your institution Buy or subscribe This is a preview of

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WITH GUILLAIN-BARRÉ SYNDROME Article Open access 26 August 2023 REFERENCES * Abrahams S _ et al_. (1997) Relation between cognitive dysfunction and pseudobulbar palsy in amyotrophic lateral

sclerosis. _J Neurol Neurosurg Psychiatry_ 62: 464–472 Article  CAS  PubMed  PubMed Central  Google Scholar  * Tandan R and Bradley WG (1985) Amyotrophic lateral sclerosis. Part 1: clinical

features, pathology, and ethical issues in management. _Ann Neurol_ 18: 271–280 Article  CAS  PubMed  Google Scholar  * Tandan R and Bradley WG (1985) Amyotrophic lateral sclerosis. Part 2:

clinical features, pathology, and ethical issues in management. _Ann Neurol_ 18: 419–431 Article  CAS  PubMed  Google Scholar  * Rosen AD (1978) Amyotrophic lateral sclerosis: clinical

features and prognosis. _Ann Neurol_ 35: 639–642 Google Scholar  * Haverkamp LJ _ et al_. (1995) Natural history of amyotrophic lateral sclerosis in a database population: validation of a

scoring system and a model for survival prediction. _Brain_ 118: 707–719 Article  PubMed  Google Scholar  * Oliver D (1996) The quality of care and symptom control—the effects on the

terminal phase of ALS/MND. _J Neurol Sci_ 139: 134–136 Article  PubMed  Google Scholar  * Desport JC _ et al_. (1999) Nutritional status is a prognostic factor for survival in ALS patients.

_Neurology_ 53: 1059–1063 Article  CAS  PubMed  Google Scholar  * Kasarskis EJ _ et al_. (1996) Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity

of death. _Am J Clin Nutr_ 63: 130–137 Article  CAS  PubMed  Google Scholar  * Bourke SC _ et al_. (2006) Effects of non-invasive ventilation on survival and quality of life in patients with

amyotrophic lateral sclerosis: a randomised controlled trial. _Lancet Neurol_ 5: 140–147 Article  PubMed  Google Scholar  * Worwood AM and Leigh PN (1998) Indicators and prevalence of

malnutrition in motor neuron disease. _Eur Neurol_ 40: 159–163 Article  CAS  PubMed  Google Scholar  * Lowe JS and Leigh N (2002) Disorders of movement and system degenerations. In

_Greenfield´s Neuropathology_, vol 2, edn 7, 325–430 (Eds Graham DI and Lantos PL) London: Arnold Google Scholar  * Strand EA _ et al_. (1996) Management of oral-pharyngeal dysphagia

symptoms in amyotrophic lateral sclerosis. _Dysphagia_ 11: 129–139 Article  CAS  PubMed  Google Scholar  * Carpenter RJ III _ et al_. (1978) The otolaryngologic presentation of amyotrophic

lateral sclerosis. _Otolaryngology_ 86: 479–484 Article  Google Scholar  * Chen A and Garrett CG (2005) Otolaryngologic presentations of amyotrophic lateral sclerosis. _Otolaryngol Head Neck

Surg_ 132: 500–504 Article  PubMed  Google Scholar  * McGuirt WF and Blalock D (1980) The otolaryngologist's role in the diagnosis and treatment of amyotrophic lateral sclerosis.

_Laryngoscope_ 90: 1496–1501 Article  CAS  PubMed  Google Scholar  * Aronson AE _ et al_. (1992) Rapid voice tremor, or “flutter,” in amyotrophic lateral sclerosis. _Ann Otol Rhinol

Laryngol_ 101: 511–518 Article  CAS  PubMed  Google Scholar  * Roth CR _ et al_. (1996) Spasmodic dysarthrophonia symptoms as initial presentation of amyotrophic lateral sclerosis. _J Voice_

10: 362–367 Article  CAS  PubMed  Google Scholar  * Kent R _ et al_. (1998) The dysarthrophonias: speech-voice profiles, related dysfunction, and neuropathology. _J Med Speech Lang Pathol_

6: 165–211 Google Scholar  * Watts CR and Vanryckeghem M (2001) Laryngeal dysfunction in amyotrophic lateral sclerosis: a review and case report. _BMC Ear Nose Throat Disord_ 1: 1 Article 

PubMed  PubMed Central  Google Scholar  * Scott A and McPhee M (2006) Multidisciplinary care: speech and language therapy. In _Palliative Care in Amyotrophic Lateral Sclerosis: from

Diagnosis to Bereavement_, edn 2, 213–227 (Eds Oliver D _ et al_.) Oxford: Oxford University Press Chapter  Google Scholar  * Yorkston K _ et al_. (1999) _Management of Motor Speech

Disorders in Children and Adults_, edn 2. Austin, TX: Pro-Ed Google Scholar  * Murphy J (2004) Perceptions of AAC by people with motor neuron disease and their communication partners.

_Augmentative and Alternative Communication_ 20: 259–271 Article  Google Scholar  * Wagner-Sonntag E and Prosiegel M (2006) Dysphagia. In _Palliative Care in Amyotrophic Lateral Sclerosis:

from Diagnosis to Bereavement_, edn 2, 95–109 (Eds Oliver D _ et al_.) Oxford: Oxford University Press Chapter  Google Scholar  * Janzen VD _ et al_. (1996) Otolaryngologic manifestations of

amyotrophic lateral sclerosis. _J Otolaryngol_ 17: 41–42 Google Scholar  * Hillel AD and Miller R (1989) Bulbar amyotrophic lateral sclerosis: patterns of progression and clinical

management. _Head Neck_ 11: 51–59 Article  CAS  PubMed  Google Scholar  * Higo R _ et al_. (2004) Longitudinal analysis of progression of dysphagia in amyotrophic lateral sclerosis. _Auris

Nasus Larynx_ 31: 247–254 Article  PubMed  Google Scholar  * Kawai S _ et al_. (2003) A study of the early stage of dysphagia in amyotrophic lateral sclerosis. _Dysphagia_ 18: 1–8 Article 

PubMed  Google Scholar  * Robbins J (1987) Swallowing in ALS and motor neuron disorders. _Neurol Clin_ 5: 213–229 Article  CAS  PubMed  Google Scholar  * Newall AR _ et al_. (1996) The

control of oral secretions in bulbar ALS/MND. _J Neurol Sci_ 139: 43–44 Article  PubMed  Google Scholar  * Hillel A _ et al_. (1999) Presentation of ALS to the otolaryngologist/head and neck

surgeon: getting to the neurologist. _Neurology_ 53: 22–25 Article  Google Scholar  * Amin MR _ et al_. (2006) Sensory testing in the assessment of laryngeal sensation in patients with

amyotrophic lateral sclerosis. _Ann Otol Rhinol Laryngol_ 115: 528–534 Article  PubMed  Google Scholar  * Power ML _ et al_. (2007) Deglutitive laryngeal closure in stroke patients. _J

Neurol Neurosurg Psychiatry_ 78: 141–146 Article  CAS  PubMed  Google Scholar  * Prosiegel M (2007) Neurogenic dysphagia [German]. _Nervenarzt_ 78: 1209–1215 Article  CAS  PubMed  Google

Scholar  * Ertekin C _ et al_. (2000) Pathophysiological mechanism of oropharyngeal dysphagia in amyotrophic lateral sclerosis. _Brain_ 123: 125–140 Article  PubMed  Google Scholar  * Sasaki

CT and Suzuki M (1997) Laryngeal spasm: a neurophysiologic redefinition. _Ann Otol Rhinol Laryngol_ 86: 150–157 Google Scholar  * Sperfeld AD _ et al_. (2005) Laryngospasm: an

underdiagnosed symptom of X-linked spinobulbar muscular atrophy. _Neurology_ 64: 753–754 Article  PubMed  Google Scholar  * Forshew DA and Bromberg MB (2003) A survey of clinicans'

practice in the symptomatic treatment of ALS. _Amyotroph Lateral Scler Other Motor Neuron Disord_ 4: 258–263 Article  PubMed  Google Scholar  * Scott A and Staios G (2004) Sensory-motor

approaches to oro-facial facilitation. In _A Practical Approach to the Management of Saliva_, edn 2, 65–73 (Eds Scott A and Johnson H) Austin, TX: Pro-Ed Google Scholar  * Beukelman D and

Ball L (2002) Improving AAC use for persons with acquired neurogenic disorders: understanding human and engineering factors. _Assist Technol_ 14: 33–44 Article  PubMed  Google Scholar  *

Kübler A _ et al_. (2005) Patients with ALS can use sensorimotor rhythms to operate a brain-computer interface. _Neurology_ 64: 1775–1777 Article  PubMed  Google Scholar  * Hardy E, Morton

Robinson N (1999) _Swallowing Disorders Treatment Manual_, edn 2. Texas: Pro-Ed Google Scholar  * Ohmae Y _ et al_. (1996) Effects of two breath-holding maneuvers on oropharyngeal swallow.

_Ann Otol Rhinol Laryngol_ 105: 123–131 Article  CAS  PubMed  Google Scholar  * Kuhlemeier KV _ et al_. (2001) Effect of liquid bolus consistency and delivery method on aspiration and

pharyngeal retention in dysphagia patients. _Dysphagia_ 16: 119–122 Article  CAS  PubMed  Google Scholar  * Hefferman C _ et al_. (2004) Nutritional management in MND/ALS patients: an

evidence based review. _Amyotroph Lateral Scler Other Motor Neuron Disord_ 5: 72–83 Article  Google Scholar  * Squires N (2006) Dysphagia management for progressive neurological conditions.

_Nurs Stand_ 20: 53–57 Article  CAS  PubMed  Google Scholar  * Norton B _ et al_. (1996) A randomised prospective comparison of percutaneous endoscopic gastrostomy and nasogastric tube

feeding after acute dysphagic stroke. _BMJ_ 312: 13–16 Article  CAS  PubMed  PubMed Central  Google Scholar  * Kasarskis EJ _ et al_. (1999) A retrospective study of percutaneous endoscopic

gastrostomy in ALS patients during the BDNF and CNTF trials. _J Neurol Sci_ 169: 118–125 Article  CAS  PubMed  Google Scholar  * Chiò A _ et al_. (2004) Percutaneous radiological

gastrostomy: a safe and effective method of nutritional tube placement in advanced ALS. _J Neurol Neurosurg Psychiatry_ 75: 645–647 Article  PubMed  PubMed Central  Google Scholar  *

Mathus-Vliegen LMH _ et al_. (1994) Percutaneous endoscopic gastrostomy in patients with amyotrophic lateral sclerosis and impaired pulmonary function. _Gastrointest Endosc_ 40: 463–469

Article  CAS  PubMed  Google Scholar  * Miller RG _ et al_. (1999) Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review). _Neurology_ 52:

1311–1323 Article  CAS  PubMed  Google Scholar  * Ludolph AC (2006) 135th ENMC International Workshop: nutrition in amyotrophic lateral sclerosis 18–20 of March 2005, Naarden, The

Netherlands. _Neuromuscul Disord_ 16: 530–538 Article  CAS  PubMed  Google Scholar  * Schrag SP _ et al_. (2007) Complications related to percutaneous endoscopic gastrostomy (PEG) tubes: a

comprehensive clinical review. _J Gastrointestin Liver Dis_ 16: 407–418 PubMed  Google Scholar  * Mazzini L _ et al_. (1995) Percutaneous endoscopic gastrostomy and enteral nutrition in

amyotrophic lateral sclerosis. _J Neurol_ 242: 695–698 Article  CAS  PubMed  Google Scholar  * Scott AG and Austin HE (1994) Nasogastric feeding in the management of severe dysphagia in

motor neuron disease. _Palliat Med_ 8: 45–49 Article  CAS  PubMed  Google Scholar  * Talmi YP _ et al_. (1989) Reduction of salivary flow in amyotrophic lateral sclerosis with Scopoderm TTS.

_Head Neck_ 11: 565 Article  CAS  PubMed  Google Scholar  * Verma A and Steele J (2006) Botulinum toxin improves sialorrhea and quality of living in bulbar amyotrophic lateral sclerosis.

_Muscle Nerve_ 34: 235–237 Article  CAS  PubMed  Google Scholar  * Andersen PM _ et al_. (2001) External radiation of the parotid glands significantly reduces drooling in patients with motor

neurone disease with bulbar paresis. _J Neurol Sci_ 191: 111–114 Article  CAS  PubMed  Google Scholar  * Kelly GS (1998) Clinical applications of N-acetylcysteine. _Altern Med Rev_ 3:

114–127 CAS  PubMed  Google Scholar  * Simmons Z (2006) Management strategies for patients with amyotrophic lateral sclerosis from diagnosis through death. _Neurologist_ 11: 257–270 Article

  Google Scholar  * Sostarko M _ et al_. (1998) Severe progression of ALS/MND after intervertebral discectomy. _J Neurol Sci_ 160: 42–46 Article  Google Scholar  * Short SO and Hillel AD

(1989) Palliative surgery in patients with bulbar amyotrophic lateral sclerosis. _Head Neck_ 11: 364–369 Article  CAS  PubMed  Google Scholar  * Hess DR (2005) Tracheostomy tubes and related

appliances. _Respir Care_ 50: 497–510 PubMed  Google Scholar  * Obholzer RJ _ et al_. (2008) An approach to the management of paroxysmal laryngospasm. _J Laryngol Otol_ 122: 57–60 Article 

CAS  PubMed  Google Scholar  * Klasner E _ et al_. (1999) Patterns of perceptual features in speakers with ALS: a preliminary study of prominence and intelligibility considerations. _J Med

Speech Lang Pathol_ 7: 117–125 Google Scholar  * Logemann JA _ et al_. (1998) Normal swallowing physiology as viewed by videofluoroscopy and videoendoscopy. _Folia Phoniatr Logop_ 50:

311–319 Article  CAS  PubMed  Google Scholar  * Wright RER and Jordan C (1997) Videofluoroscopic evaluation of dysphagia in motor neuron disease with modified barium swallow. _Palliat Med_

11: 44–48 Article  CAS  PubMed  Google Scholar  * Tsokos M _ et al_. (1998) Barium aspiration with fatal outcome [German]. _Aktuelle Radiol_ 8: 201–203 CAS  PubMed  Google Scholar  *

Miyazawa T _ et al_. (1990) Effect of water-soluble contrast medium on the lung in rats. Comparison of iotrolan, iopamidol, and diatrizoate. _Invest Radiol_ 25: 999–1003 Article  CAS  PubMed

  Google Scholar  * Rosenbek JC _ et al_. (1996) A penetration–aspiration scale. _Dysphagia_ 11: 93–98 Article  CAS  PubMed  Google Scholar  * Langmore SE (2001) _Endoscopic Evaluation and

Treatment of Swallowing Disorders_. New York: Thieme Google Scholar  * Doggett DL _ et al_. (2002) Recent developments in diagnosis and intervention for aspiration and dysphagia in stroke

and other neuromuscular disorders. _Curr Atheroscler Rep_ 4: 311–318 Article  PubMed  Google Scholar  * Leder SB _ et al_. (2004) Use of fiberoptic endoscopic evaluation of swallowing (FEES)

in patients with amyotrophic lateral sclerosis. _Dysphagia_ 19: 177–181 Article  PubMed  Google Scholar  Download references 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. AUTHOR INFORMATION AUTHORS AND

AFFILIATIONS * P Kühnlein and H-J Gdynia are Residents in the Department of Neurology, A-D Sperfeld is an Associate Professor in the Department of Neurology and Head of the Department for

Neuromuscular Disorders, B Lindner-Pfleghar is a Speech Therapist in the Department of Neurology, AC Ludolph is Director of the Department of Neurology, and A Riecker is an Associate

Professor in the Department of Neurology and Head of the Department for Swallowing Disorders, all at the University of Ulm, Ulm, Germany., Peter Kühnlein, Hans-Jürgen Gdynia, Anne-Dorte

Sperfeld, Beate Lindner-Pfleghar, Albert Christian Ludolph & Axel Riecker * M Prosiegel is Director of the Department of Neurology at the Rehabilitation Clinic Bad Heilbrunn, Bad

Heilbrunn, Germany., Mario Prosiegel Authors * Peter Kühnlein View author publications You can also search for this author inPubMed Google Scholar * Hans-Jürgen Gdynia View author

publications You can also search for this author inPubMed Google Scholar * Anne-Dorte Sperfeld View author publications You can also search for this author inPubMed Google Scholar * Beate

Lindner-Pfleghar View author publications You can also search for this author inPubMed Google Scholar * Albert Christian Ludolph View author publications You can also search for this author

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this author inPubMed Google Scholar CORRESPONDING AUTHOR Correspondence to Axel Riecker. ETHICS DECLARATIONS COMPETING INTERESTS The authors declare no competing financial interests. RIGHTS

AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE 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 Download citation * Received: 04 February 2008 * Accepted: 12 May 2008 * Published: 17 June 2008 *

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