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Trigeminal Neuralgia in Emergency Medicine

  • Author: J Stephen Huff, MD, FACEP; Chief Editor: Robert E O'Connor, MD, MPH  more...
 
Updated: Oct 22, 2015
 

Background

Trigeminal neuralgia (TN), also known as tic douloureux, is a pain syndrome often recognizable by the patient's history alone. Trigeminal neuralgia is characterized by facial pain often accompanied by a brief facial spasm or tic. Pain distribution is unilateral and follows the sensory distribution of cranial nerve V, typically radiating to the maxillary (V2) or mandibular (V3) area. At times, both distributions are affected. Physical examination will usually eliminate alternative diagnoses. Signs of cranial nerve dysfunction or other neurologic abnormality exclude the diagnosis of classic trigeminal neuralgia and suggest that pain may be secondary to a structural lesion. Nomenclature is nonintuitive. Classic trigeminal neuralgia includes all cases without established etiology after investigation, as well as those with potential microvascular compression of the fifth cranial nerve. In symptomatic trigeminal neuralgia, the pain syndrome is secondary to tumor, multiplesclerosis, orother structural abnormalities.[1]

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Pathophysiology

The mechanism of pain production remains controversial. One theory suggests that peripheral injury or disease of the trigeminal nerve increases afferent firing in the nerve; failure of central inhibitory mechanisms may be involved as well. Pain is perceived when nociceptive neurons in a trigeminal nucleus involve thalamic relay neurons. An abnormal vascular course of the superior cerebellar artery is often cited as the cause, as well as other small arteries or veins compressing the facial nerve. In about 85% of cases, no lesion is identified, even after extensive investigations, and the etiology is labeled idiopathic by default.

Aneurysms, tumors, chronic meningeal inflammation, or other lesions may irritate trigeminal nerve roots along the pons, causing symptomatic trigeminal neuralgia. Uncommonly, an area of demyelination from multiple sclerosis may be the precipitant. Lesions of the entry zone of the trigeminal roots within the pons may cause a similar pain syndrome.

An area of demyelination is shown in the image below.

Microscopic demonstration of demyelination in prim Microscopic demonstration of demyelination in primary trigeminal neuralgia. A tortuous axon is surrounded by abnormally discontinuous myelin. Electron microscope, 3, 300 X.

Infrequently, adjacent dental fillings composed of dissimilar metals may trigger attacks,[2] and an atypical case has been reported following tongue piercing. A case report of trigeminal neuralgia in a patient with spontaneous intracranial hypotension has been reported; both conditions resolved following surgical treatment of a cervical root sleeve dural defect.[3]

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Epidemiology

Frequency

Trigeminal neuralgia (TN) is uncommon, with an estimated prevalence of 155 cases per million persons.

Mortality/Morbidity

No mortality is associated with idiopathic trigeminal neuralgia (TN), although secondary depression is common if a chronic pain syndrome evolves. In rare cases, pain may be so frequent that oral nutrition is impaired.

In symptomatic or secondary trigeminal neuralgia, morbidity or mortality relates to the underlying cause of the pain syndrome.

Age

Development of trigeminal neuralgia in a young person suggests the possibility of multiple sclerosis.

Idiopathic trigeminal neuralgia typically occurs in patients in the sixth decade of life, but it may occur at any age.

Symptomatic or secondary trigeminal neuralgia tends to occur in younger patients.

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Contributor Information and Disclosures
Author

J Stephen Huff, MD, FACEP Professor of Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia School of Medicine

J Stephen Huff, MD, FACEP is a member of the following medical societies: American Academy of Neurology, American College of Emergency Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Robert E O'Connor, MD, MPH Professor and Chair, Department of Emergency Medicine, University of Virginia Health System

Robert E O'Connor, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American Association for Physician Leadership, American Heart Association, Medical Society of Delaware, Society for Academic Emergency Medicine, Wilderness Medical Society, American Medical Association, National Association of EMS Physicians

Disclosure: Nothing to disclose.

Additional Contributors

Theodore J Gaeta, DO, MPH, FACEP Clinical Associate Professor, Department of Emergency Medicine, Weill Cornell Medical College; Vice Chairman and Program Director of Emergency Medicine Residency Program, Department of Emergency Medicine, New York Methodist Hospital; Academic Chair, Adjunct Professor, Department of Emergency Medicine, St George's University School of Medicine

Theodore J Gaeta, DO, MPH, FACEP is a member of the following medical societies: American College of Emergency Physicians, New York Academy of Medicine, Society for Academic Emergency Medicine, Council of Emergency Medicine Residency Directors, Clerkship Directors in Emergency Medicine, Alliance for Clinical Education

Disclosure: Nothing to disclose.

Acknowledgements

Tom Scaletta, MD President, Smart-ER (http://smart-er.net); Chair, Department of Emergency Medicine, Edward Hospital; Past-President, American Academy of Emergency Medicine

Tom Scaletta, MD is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

References
  1. [Guideline] Cruccu G, Gronseth G, Alksne J, et al. AAN-EFNS guidelines on trigeminal neuralgia management. Eur J Neurol. 2008 Oct. 15(10):1013-28. [Medline].

  2. Cheshire WP Jr. The shocking tooth about trigeminal neuralgia. N Engl J Med. 2000 Jun 29. 342(26):2003. [Medline].

  3. Cheshire WP Jr, Wharen RE Jr. Trigeminal neuralgia in a patient with spontaneous intracranial hypotension. Headache. 2009 May. 49(5):770-3. [Medline].

  4. Gronseth G, Cruccu G, Alksne J, Argoff C, Brainin M, Burchiel K, et al. Practice parameter: the diagnostic evaluation and treatment of trigeminal neuralgia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the European Federation of Neurological Societies. Neurology. 2008 Oct 7. 71(15):1183-90. [Medline].

  5. Cheshire WP. Fosphenytoin: an intravenous option for the management of acute trigeminal neuralgia crisis. J Pain Symptom Manage. 2001 Jun. 21 (6):506-10. [Medline].

  6. Vargas A, Thomas K. Intravenous fosphenytoin for acute exacerbation of trigeminal neuralgia: case report and literature review. Ther Adv Neurol Disord. 2015 Jul. 8 (4):187-8. [Medline].

  7. Knafo H, Kenny B, Mathieu D. Trigeminal neuralgia: outcomes after gamma knife radiosurgery. Can J Neurol Sci. 2009 Jan. 36(1):78-82. [Medline].

  8. Niki Y, Kanai A, Hoshi K, Okamoto H. Immediate Analgesic Effect of 8% Lidocaine Applied to the Oral Mucosa in Patients with Trigeminal Neuralgia. Pain Med. 2014 Feb 7. [Medline].

  9. Zhang J, Yang M, Zhou M, He L, Chen N, Zakrzewska JM. Non-antiepileptic drugs for trigeminal neuralgia. Cochrane Database Syst Rev. 2013 Dec 3. 12:CD004029. [Medline].

  10. Bennetto L, Patel NK, Fuller G. Trigeminal neuralgia and its management. BMJ. 2007 Jan 27. 334(7586):201-5. [Medline].

  11. Chole R, Patil R, Degwekar SS, Bhowate RR. Drug treatment of trigeminal neuralgia: a systematic review of the literature. J Oral Maxillofac Surg. 2007 Jan. 65(1):40-5. [Medline].

  12. Gomez-Arguelles JM, Dorado R, Sepulveda JM, Herrera A, Arrojo FG, Aragon E, et al. Oxcarbazepine monotherapy in carbamazepine-unresponsive trigeminal neuralgia. J Clin Neurosci. 2008 May. 15(5):516-9. [Medline].

 
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Microscopic demonstration of demyelination in primary trigeminal neuralgia. A tortuous axon is surrounded by abnormally discontinuous myelin. Electron microscope, 3, 300 X.
MRI with high resolution on the pons demonstrating the trigeminal nerve root. In this case, the patient with trigeminal neuralgia has undergone gamma-knife therapy, and the left-sided treated nerve (arrow) is enhanced by gadolinium.
 
 
 
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