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Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis

Nature Clinical Practice Neurology volume 4, pages 366–374 (2008)Cite this article

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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References

  1. 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 

  2. 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 

  3. 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 

  4. Rosen AD (1978) Amyotrophic lateral sclerosis: clinical features and prognosis. Ann Neurol 35: 639–642

    Google Scholar 

  5. 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 

  6. 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 

  7. 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 

  8. 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 

  9. 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 

  10. 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 

  11. 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 

  12. Strand EA et al. (1996) Management of oral-pharyngeal dysphagia symptoms in amyotrophic lateral sclerosis. Dysphagia 11: 129–139

    Article  CAS  PubMed  Google Scholar 

  13. Carpenter RJ III et al. (1978) The otolaryngologic presentation of amyotrophic lateral sclerosis. Otolaryngology 86: 479–484

    Article  Google Scholar 

  14. Chen A and Garrett CG (2005) Otolaryngologic presentations of amyotrophic lateral sclerosis. Otolaryngol Head Neck Surg 132: 500–504

    Article  PubMed  Google Scholar 

  15. 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 

  16. 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 

  17. 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 

  18. Kent R et al. (1998) The dysarthrophonias: speech-voice profiles, related dysfunction, and neuropathology. J Med Speech Lang Pathol 6: 165–211

    Google Scholar 

  19. 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 

  20. 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 

  21. Yorkston K et al. (1999) Management of Motor Speech Disorders in Children and Adults, edn 2. Austin, TX: Pro-Ed

    Google Scholar 

  22. 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 

  23. 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 

  24. Janzen VD et al. (1996) Otolaryngologic manifestations of amyotrophic lateral sclerosis. J Otolaryngol 17: 41–42

    Google Scholar 

  25. 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 

  26. 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 

  27. 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 

  28. Robbins J (1987) Swallowing in ALS and motor neuron disorders. Neurol Clin 5: 213–229

    Article  CAS  PubMed  Google Scholar 

  29. Newall AR et al. (1996) The control of oral secretions in bulbar ALS/MND. J Neurol Sci 139: 43–44

    Article  PubMed  Google Scholar 

  30. 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 

  31. 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 

  32. Power ML et al. (2007) Deglutitive laryngeal closure in stroke patients. J Neurol Neurosurg Psychiatry 78: 141–146

    Article  CAS  PubMed  Google Scholar 

  33. Prosiegel M (2007) Neurogenic dysphagia [German]. Nervenarzt 78: 1209–1215

    Article  CAS  PubMed  Google Scholar 

  34. Ertekin C et al. (2000) Pathophysiological mechanism of oropharyngeal dysphagia in amyotrophic lateral sclerosis. Brain 123: 125–140

    Article  PubMed  Google Scholar 

  35. Sasaki CT and Suzuki M (1997) Laryngeal spasm: a neurophysiologic redefinition. Ann Otol Rhinol Laryngol 86: 150–157

    Google Scholar 

  36. Sperfeld AD et al. (2005) Laryngospasm: an underdiagnosed symptom of X-linked spinobulbar muscular atrophy. Neurology 64: 753–754

    Article  PubMed  Google Scholar 

  37. 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 

  38. 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 

  39. 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 

  40. 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 

  41. Hardy E, Morton Robinson N (1999) Swallowing Disorders Treatment Manual, edn 2. Texas: Pro-Ed

    Google Scholar 

  42. 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 

  43. 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 

  44. 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 

  45. Squires N (2006) Dysphagia management for progressive neurological conditions. Nurs Stand 20: 53–57

    Article  CAS  PubMed  Google Scholar 

  46. 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 

  47. 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 

  48. 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 

  49. 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 

  50. 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 

  51. 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 

  52. 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 

  53. 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 

  54. 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 

  55. 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 

  56. 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 

  57. 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 

  58. Kelly GS (1998) Clinical applications of N-acetylcysteine. Altern Med Rev 3: 114–127

    CAS  PubMed  Google Scholar 

  59. Simmons Z (2006) Management strategies for patients with amyotrophic lateral sclerosis from diagnosis through death. Neurologist 11: 257–270

    Article  Google Scholar 

  60. Sostarko M et al. (1998) Severe progression of ALS/MND after intervertebral discectomy. J Neurol Sci 160: 42–46

    Article  Google Scholar 

  61. 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 

  62. Hess DR (2005) Tracheostomy tubes and related appliances. Respir Care 50: 497–510

    PubMed  Google Scholar 

  63. Obholzer RJ et al. (2008) An approach to the management of paroxysmal laryngospasm. J Laryngol Otol 122: 57–60

    Article  CAS  PubMed  Google Scholar 

  64. 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 

  65. 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 

  66. 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 

  67. Tsokos M et al. (1998) Barium aspiration with fatal outcome [German]. Aktuelle Radiol 8: 201–203

    CAS  PubMed  Google Scholar 

  68. 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 

  69. Rosenbek JC et al. (1996) A penetration–aspiration scale. Dysphagia 11: 93–98

    Article  CAS  PubMed  Google Scholar 

  70. Langmore SE (2001) Endoscopic Evaluation and Treatment of Swallowing Disorders. New York: Thieme

    Google Scholar 

  71. 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 

  72. 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 

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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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Authors and Affiliations

  1. 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

  2. M Prosiegel is Director of the Department of Neurology at the Rehabilitation Clinic Bad Heilbrunn, Bad Heilbrunn, Germany.,

    Mario Prosiegel

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  2. Hans-Jürgen Gdynia
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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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