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Upper Motor Neuron Diseases and Laryngeal Presentations

  • Neurolaryngology (AJ McWhorter and L Adkins, Section Editors)
  • Published:
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Abstract

Purpose of Review

The purpose of this manuscript is to review laryngeal manifestations of upper motor neuron disease. A review of the relevant neuroanatomy is performed as well as a description of common laryngeal signs of upper motor neuron disease and the disease entities that cause them.

Recent Findings

Upper motor neuron lesions must occur in the brainstem or in the bilateral cerebral hemisphere in order to cause signs and symptoms in the larynx.

Summary

The presence of bilateral vocal fold paralysis or paresis with concurrent signs of myoclonus, tremor, rigidity, hyperactive gag reflex, and/or laryngospasm episodes should prompt an investigation for possible medullary brainstem or bilateral cerebral cortex pathology.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Felten DL, O’Banion MK, Maida MS. Motor systems. In: Felten DL, O’Banion MK, Maida MS, editors. Netter’s atlas of neuroscience. 3rd ed. Philadelphia: Elsevier; 2016. p. 391–420.

    Chapter  Google Scholar 

  2. Rea P. Spinal tracts: descending - motor pathways. In: Rea P, editor. Essential clinical anatomy of the nervous system. London: Elsevier; 2015. p. 161–76.

    Chapter  Google Scholar 

  3. Johns P. Sensory and motor pathways. In: Johns P, editor. Clinical neuroscience. London: Churchill Livingston Elsevier; 2014. p. 49–59.

    Chapter  Google Scholar 

  4. Huang CY, Broe G. Isolated facial palsy: a new lacunar syndrome. J Neurol Neurosurg Psychiatry. 1984;47:84–6.

    Article  CAS  Google Scholar 

  5. Besson G, Bogousslavsky J, Regli F, Maeder P. Acute pseudobulbar or suprabulbar palsy. Arch Neurol. 1991;48:501–7.

    Article  CAS  Google Scholar 

  6. . Bach JR, Upadhyaya N. Association of need for tracheotomy with decreasing mechanical ex-sufflation flows in amyotrophic lateral sclerosis. Am J Phys Med Rehabil. 2018;97:e20–2. https://doi.org/10.1097/PHM.0000000000000755This article explores the use of in-exsufflation flows in helping to determine when a patient with amyotrophic lateral sclerosis would benefit from tracheotomy.

    Article  PubMed  Google Scholar 

  7. Factor SA, Weiner WJ. Hyperkinetic movement disorders. In: Weiner WJ, Goetz CG, editors. Neurology for the non-neurologist. 3rd ed. Philadelphia: JB Lippincott Company; 1994. p. 121–45.

    Google Scholar 

  8. Myoclonus fact sheet, NINDS, Publication date July 2012. NIH Publication No. 12–4793. https://www.ninds.nih.gov/disorders/patient-caregiver-education/fact-sheets/myoclonus-fact-sheet.

  9. Ivanhoe CB, Reistetter TA. Spasticity: the misunderstood part of the upper motor neuron syndrome. Am J Phys Med Rehabil. 2004;83:S3–9.

    Article  Google Scholar 

  10. Wasdman SD. The pyramidal system. In: Waldman SD, editor. Pain review. Philadelphia: Elsevier; 2009. p. 178–9.

    Chapter  Google Scholar 

  11. Dystonias fact sheet, NINDS, Publication date January 2012.NIH Publication No. 12–717. https://www.ninds.nih.gov/disorders/patient-caregiver-education/fact-sheets/dystonias-fact-sheet.

  12. Parkinson’s disease: hope through research, NINDS, Publication date December 2014. NIH Publication No. 15–139. https://www.ninds.nih.gov/disorders/patient-caregiver-education/hope-through-research/parkinsons-disease-hope-through-research.

  13. Van der Graaff MM, Grolman W, Westermann EJ, Boogardt HC, et al. Vocal cord dysfunction in amyotrophic lateral sclerosis. Arch Neurol. 2009;66:1329–33.

    PubMed  Google Scholar 

  14. Simonds AK. Progress in respiratory management of bulbar complications of motor neuron disease/amyotrophic lateral sclerosis. Thorax. 2016;72:0:1–3. https://doi.org/10.1136/thoraxjnl-2016-208919.

    Article  Google Scholar 

  15. Watts CR, Vanryckeghem M. Laryngeal dysfunction in amyotrophic lateral sclerosis: a review and case report. BMC Ear Nose Throat Disord. 2001;1:1.

    Article  Google Scholar 

  16. Motor neuron diseases fact sheet, NINDS, Publication date: August2019. NIH Publication No. 19-NS-5371. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Motor-Neuron-Diseases-Fact-Sheet

  17. Becker A, Hardmeier M, Steck AJ, Czaplinski A. Primary lateral sclerosis presenting with isolated progressive pseudobulbar syndrome. Eur J Neurol. 2007;14:e3. https://doi.org/10.1111/j.1468-1331.2007.01699.x.

    Article  CAS  PubMed  Google Scholar 

  18. Motomura S, Tabira T, Kuroiwa Y. A clinical comparative study of multiple sclerosis and neuro-Behcet’s syndrome. J Neurol Neurosurg Psychiatry. 1980;43:210–3.

    Article  CAS  Google Scholar 

  19. . Baiardi S, Capellari S, Bartoletti SA, Parchi P. Unusual clinical presentations challenging the early clinical diagnosis of Creutzfeldt-Jakob disease. J Alzheimers Dis. 2018;64:1051–65. https://doi.org/10.3233/JAD-180123This article highlights the role of neurologic consultation in the early evaluation and management of patients with clinical presentation of pseudobulbar palsy, of which the otolaryngologist may be the first clinician to evaluate and notice the sentinel signs.

    Article  CAS  PubMed  Google Scholar 

  20. . Oz Tuncer G, Teber S, Kutluk MG, Albayrak P, Deda G. Hashimoto’s encephalopathy presenting as pseudobulbar palsy. Childs Nerv Syst. 2018;34:1251–4. https://doi.org/10.1007/s00381-018-3720-2This article highlights the role of neurologic consultation in the early evaluation and management of patients with clinical presentation of pseudobulbar palsy, even in the pediatric population, where the otolaryngologist may be the first clinician to evaluate and notice the sentinel signs.

    Article  PubMed  Google Scholar 

  21. Nicastro N, Ghika J, Pollak P, Horvat J. Pseudobulbar palsy due to deep-brain stimulation of the thalamic ventral intermediate nuclei. Clin Neurol Neurosurg. 2015;133:61–3. https://doi.org/10.1016/j.clineuro.2015.03.013.

    Article  PubMed  Google Scholar 

  22. Pearce JM. Central pontine myelinolysis. Eur Neurol. 2009;61:59–62. https://doi.org/10.1159/000175124.

    Article  CAS  PubMed  Google Scholar 

  23. McCormick WE, Lee JH. Pseudobulbar palsy caused by a large petroclival meningioma: report of two cases. Skull Base. 2002;12:67–71.

    Article  Google Scholar 

  24. Mohapatra MK, Sethy G, Mohanty SC. Pseudobulbar paralysis—a sequelae of cerebral malaria. J Assoc Physicians India. 2004;52:324–5.

    CAS  PubMed  Google Scholar 

  25. Wisoff JH, Epstein FJ. Pseudobulbar palsy after posterior fossa operation in children. Neurosurgery. 1984;15:707–9.

    Article  CAS  Google Scholar 

  26. Solomon RA, Correll JW. Pseudobulbar palsy after carotid endarterectomy. Neurosurgery. 1985;17:527–8.

    Article  CAS  Google Scholar 

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Correspondence to Yolanda D. Heman-Ackah.

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This article is part of the Topical collection on Neurolaryngology

Electronic supplementary material

ESM 1

Video 1. Resting tremor in the larynx. This video shows a rhythmic tremor of the laryngeal muscles at rest during quiet respiration. (Used with permission from Yolanda D. Heman-Ackah.) (WMV 1017 kb)

ESM 2

Video 2. Laryngeal intention tremor. This video shows absence of tremor at rest and the presence of a rhythmic tremor of the laryngeal muscles with sustained adduction of the vocal folds during phonation. (Used with permission from Yolanda D. Heman-Ackah.) (WMV 2070 kb)

ESM 3

Video 3. Laryngeal Myoclonus. This video shows myoclonic jerking movements of the laryngeal and pharyngeal constrictor muscles at rest. (Used with permission from Yolanda D. Heman-Ackah.) (WMV 664 kb)

ESM 4

Video 4. Laryngeal cogwheeling. This video shows rigidity in the laryngeal muscles as they attempt adduction following abduction. (Used with permission from Yolanda D. Heman-Ackah.) (MPG 2401 kb)

ESM 5

Video 5. Laryngeal dysdiadokinesis. This video shows difficulty alternating quickly between abduction and adduction of the vocal folds. (Used with permission from Yolanda D. Heman-Ackah.) (MPG 5194 kb)

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Heman-Ackah, Y.D. Upper Motor Neuron Diseases and Laryngeal Presentations. Curr Otorhinolaryngol Rep 8, 230–235 (2020). https://doi.org/10.1007/s40136-020-00292-9

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