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.
Similar content being viewed by others
References
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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.
Rea P. Spinal tracts: descending - motor pathways. In: Rea P, editor. Essential clinical anatomy of the nervous system. London: Elsevier; 2015. p. 161–76.
Johns P. Sensory and motor pathways. In: Johns P, editor. Clinical neuroscience. London: Churchill Livingston Elsevier; 2014. p. 49–59.
Huang CY, Broe G. Isolated facial palsy: a new lacunar syndrome. J Neurol Neurosurg Psychiatry. 1984;47:84–6.
Besson G, Bogousslavsky J, Regli F, Maeder P. Acute pseudobulbar or suprabulbar palsy. Arch Neurol. 1991;48:501–7.
. 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.
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.
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.
Ivanhoe CB, Reistetter TA. Spasticity: the misunderstood part of the upper motor neuron syndrome. Am J Phys Med Rehabil. 2004;83:S3–9.
Wasdman SD. The pyramidal system. In: Waldman SD, editor. Pain review. Philadelphia: Elsevier; 2009. p. 178–9.
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.
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.
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.
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.
Watts CR, Vanryckeghem M. Laryngeal dysfunction in amyotrophic lateral sclerosis: a review and case report. BMC Ear Nose Throat Disord. 2001;1:1.
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
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.
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.
. 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.
. 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.
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.
Pearce JM. Central pontine myelinolysis. Eur Neurol. 2009;61:59–62. https://doi.org/10.1159/000175124.
McCormick WE, Lee JH. Pseudobulbar palsy caused by a large petroclival meningioma: report of two cases. Skull Base. 2002;12:67–71.
Mohapatra MK, Sethy G, Mohanty SC. Pseudobulbar paralysis—a sequelae of cerebral malaria. J Assoc Physicians India. 2004;52:324–5.
Wisoff JH, Epstein FJ. Pseudobulbar palsy after posterior fossa operation in children. Neurosurgery. 1984;15:707–9.
Solomon RA, Correll JW. Pseudobulbar palsy after carotid endarterectomy. Neurosurgery. 1985;17:527–8.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of Interest
The author declares that she has no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
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)
Rights and permissions
About this article
Cite this article
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
Published:
Issue Date:
DOI: https://doi.org/10.1007/s40136-020-00292-9