eMedicine Specialties > Ophthalmology > Neurologic Disorders

Trigeminal Neuralgia

Author: Marc E Lenaerts, MD, Clerkship Director, Assistant Professor, Department of Neurology, University of Oklahoma
Coauthor(s): James R Couch, MD, PhD, FACP, Professor of Neurology, University of Oklahoma Health Sciences Center
Contributor Information and Disclosures

Updated: Mar 17, 2006

Introduction

Background

First detailed in the 18th century, trigeminal neuralgia is a severe, lightninglike pain in the trigeminal nerve territory that can be triggered by various stimuli (eg, touch).

Diagnostic criteria for idiopathic trigeminal neuralgia by the International Headache Society are as follows:

  • Paroxysmal attacks of facial or frontal pain occur, lasting a few seconds to less than 2 minutes.
  • Pain has at least 4 of the following characteristics:
    • Distribution along 1 or more divisions of the trigeminal nerve
    • Sudden, intense, sharp, superficial, stabbing, or burning in quality
    • Severe pain intensity
    • Precipitation from trigger areas or by certain daily activities (eg, eating, talking, washing the face, cleaning the teeth)
    • No symptoms between paroxysms
  • No neurologic deficit is present.
  • Attacks are stereotyped in the individual patient.
  • Other causes of facial pain are excluded by history, physical examination, and special investigations (when necessary). In symptomatic cases, a persistence of aching can occur between paroxysms, as well as signs of sensory impairment in the trigeminal division. Then, a cause is demonstrated by appropriate investigation.

Pathophysiology

Trigeminal neuralgia is the prototype of neuropathic pain, meaning that the pain mechanisms themselves are altered. Evidence of both small and large fiber damage is present, as suggested by the potential for vibration to trigger an attack. Demyelination of the nerve, primary or secondary, leads to uncontrolled firing of small unmyelinated trigeminal nerve fibers. This occurs, in part, because of the lack of inhibitory inputs from large myelinated nerve fibers. However, features also suggest a partly central mechanism (eg, delay between stimulation and pain, refractory period). The central abnormalities are still poorly understood. See Image 1.

Frequency

United States

Prevalence in the United States is approximately 1.5 cases per 10,000 population. Incidence in the United States is about 15,000 cases per year. Although a questionable family clustering exists, trigeminal neuralgia most likely is multifactorial.

International

No geographic tendency exists.

Mortality/Morbidity

Mortality is virtually nonexistent; however, the severity of the pain may lead to suicide. This disease has no significant morbidity, except for the burden of long-term pain and possible reactive depression.

Race

No racial differences regarding this disease have been reported.

Sex

Females are affected twice as often as males.

Age

Most cases develop in patients older than 50 years, but occasional reports of pediatric cases indicate a large range of age at onset.

Clinical

History

Although it may be interrupted by remissions of a few months, trigeminal neuralgia is a long-term condition, consisting of episodic headache. This disease has the typical characteristics of neuropathic pain.

  • The pain is lightninglike and hardly lasts for longer than a second. Because multiple bouts frequently follow each other, the patient often misleadingly describes a continuous pain. A refractory period that can be as short as a couple of seconds occurs. The intense pain is followed by a bothersome sensation in the area. If prominent, the painful sensation is indicative of a potential underlying lesion (eg, tumor) in the posterior fossa.
  • In most cases, pain is located in V3 or V2; it is located rarely in both and exceptionally in V1. Pain is unilateral and does not shift sides. Although very rare, bilateral cases have been described.
  • Different stimuli can trigger pain, often consistent in each patient. These stimuli include the following:
    • Touching or applying heat or cold to the cheek or gum
    • Chewing, yawning, or talking
    • Wind blowing in the face
    • Gustatory stimuli and vibration (In experimental studies, these stimuli have been described, demonstrating the role of large myelinated fibers in the pathophysiology.)

Physical

Except for a trigger zone, usually in the face, general and neurologic examinations should be normal. Although they may be observed transitorily in idiopathic trigeminal neuralgia, hypesthesia or dysesthesia in the face should be considered part of the secondary forms.

Causes

Trigeminal neuralgia is divided into 2 categories, idiopathic and secondary. Secondary forms can have multiple origins, as outlined below. (This list is not exclusive.)

  • Tumor
    • Acoustic neurinoma
    • Chordoma at the level of the clivus
    • Pontine glioma or glioblastoma
    • Epidermoid
    • Metastases
    • Lymphoma
  • Vascular
  • Pontine infarct
  • Arteriovenous malformation in the vicinity
  • Persistence of a primitive trigeminal artery
  • Pulsatile compression by the adjacent superior cerebellar artery (more rarely, anteroinferior artery)
  • Inflammatory
  • Multiple sclerosis (MS)
  • Sarcoidosis
  • Lyme disease neuropathy
  • Paraneoplastic (possibly)

More on Trigeminal Neuralgia

Overview: Trigeminal Neuralgia
Differential Diagnoses & Workup: Trigeminal Neuralgia
Treatment & Medication: Trigeminal Neuralgia
Follow-up: Trigeminal Neuralgia
Multimedia: Trigeminal Neuralgia
References
[ CLOSE WINDOW ]

References

  1. Al-Din AS, Mir R, Davey R, et al. Trigeminal cephalgias and facial pain syndromes associated with autonomic dysfunction. Cephalalgia. Aug 2005;25(8):605-11. [Medline].

  2. Baker KA, Taylor JW, Lilly GE. Treatment of trigeminal neuralgia: use of baclofen in combination with carbamazepine. Clin Pharm. Jan-Feb 1985;4(1):93-6. [Medline].

  3. Blom S. Trigeminal neuralgia: Its treatment with a new anticonvulsant drug. Lancet. 1962;1:839-40.

  4. Brabant S, Van Zundert J, Van Buyten JP. Pulsed radiofrequency treatment of the gasserian ganglion in patients with essential trigeminus neuralgia: A retrospective study. Proceedings of the World Pain Congress;. 2000;San Francisco, CA.

  5. Browne L. Radiofrequency lesioning of the trigeminal ganglion for the treatment of trigeminal neuralgia. Ir Med J. Mar 1985;78(3):68-71. [Medline].

  6. Cruccu G, Biasiotta A, Galeotti F, et al. Diagnostic accuracy of trigeminal reflex testing in trigeminal neuralgia. Neurology. Jan 10 2006;66(1):139-41. [Medline].

  7. Dalessio DJ. Diagnosis and treatment of cranial neuralgias. Med Clin North Am. May 1991;75(3):605-15. [Medline].

  8. Deinsberger R, Tidstrand J. Linac radiosurgery as a tool in neurosurgery. Neurosurg Rev. Apr 2005;28(2):79-88; discussion 89-90, 91. [Medline].

  9. Dubner R, Sharav Y, Gracely RH, Price DD. Idiopathic trigeminal neuralgia: sensory features and pain mechanisms. Pain. Oct 1987;31(1):23-33. [Medline].

  10. Eide PK, Rabben T. Trigeminal neuropathic pain: pathophysiological mechanisms examined by quantitative assessment of abnormal pain and sensory perception. Neurosurgery. Nov 1998;43(5):1103-10. [Medline].

  11. Evans RW, Graff-Radford SB, Bassiur JP. Pretrigeminal neuralgia. Headache. Mar 2005;45(3):242-4. [Medline].

  12. Fromm GH. Pathophysiology of trigeminal neuralgia. In: Fromm G, Sessle B, eds. Trigeminal Neuralgia: Current Concepts Regarding Pathogenesis and Treatment. Woburn, MA: Butterworth;1991:105-22.

  13. Fromm GH, Terrence CF, Chattha AS. Baclofen in the treatment of trigeminal neuralgia: double-blind study and long-term follow-up. Ann Neurol. Mar 1984;15(3):240-4. [Medline].

  14. Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders: 2nd edition. Cephalalgia. 2004;24 Suppl 1:9-160. [Medline].

  15. Hess B, Oberndorfer S, Urbanits S, et al. Trigeminal neuralgia in two patients with glioblastoma. Headache. Oct 2005;45(9):1267-70. [Medline].

  16. Khan OA. Gabapentin relieves trigeminal neuralgia in multiple sclerosis patients. Neurology. Aug 1998;51(2):611-4. [Medline].

  17. Khedr EM, Kotb H, Kamel NF, et al. Longlasting antalgic effects of daily sessions of repetitive transcranial magnetic stimulation in central and peripheral neuropathic pain. J Neurol Neurosurg Psychiatry. Jun 2005;76(6):833-8. [Medline].

  18. Kondziolka D, Perez B, Flickinger JC, et al. Gamma knife radiosurgery for trigeminal neuralgia: results and expectations. Arch Neurol. Dec 1998;55(12):1524-9. [Medline].

  19. Kondziolka D. Functional radiosurgery. Neurosurgery. Jan 1999;44(1):12-20; discussion 20-2. [Medline].

  20. Olson S, Atkinson L, Weidmann M. Microvascular decompression for trigeminal neuralgia: recurrences and complications. J Clin Neurosci. Sep 2005;12(7):787-9. [Medline].

  21. Pollock BE, Ecker RD. A prospective cost-effectiveness study of trigeminal neuralgia surgery. Clin J Pain. Jul-Aug 2005;21(4):317-22. [Medline].

  22. Rose FC. Trigeminal neuralgia. Arch Neurol. Sep 1999;56(9):1163-4. [Medline].

  23. Tanaka T, Morimoto Y, Shiiba S, et al. Utility of magnetic resonance cisternography using three-dimensional fast asymmetric spin-echo sequences with multiplanar reconstruction: the evaluation of sites of neurovascular compression of the trigeminal nerve. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Aug 2005;100(2):215-25. [Medline].

  24. Truini A, Galeotti F, Cruccu G. New insight into trigeminal neuralgia. J Headache Pain. Sep 2005;6(4):237-9. [Medline].

  25. Turk U, Ilhan S, Alp R, et al. Botulinum toxin and intractable trigeminal neuralgia. Clin Neuropharmacol. Jul-Aug 2005;28(4):161-2. [Medline].

  26. Vincent M. SUNCT, lacrimation, and trigeminal neuralgia. Cephalalgia. Mar 1998;18(2):71. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

tic douloureux

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Marc E Lenaerts, MD, Clerkship Director, Assistant Professor, Department of Neurology, University of Oklahoma
Marc E Lenaerts, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Merck and OrthoMcNeil Neurologics Honoraria Speaking and teaching

Coauthor(s)

James R Couch, MD, PhD, FACP, Professor of Neurology, University of Oklahoma Health Sciences Center
James R Couch, MD, PhD, FACP is a member of the following medical societies: American Academy of Neurology, American Geriatrics Society, American Headache Society, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, American Stroke Association, and United Council of Neurologic Subspecialties, Certification in Headache Medicine
Disclosure: Nothing to disclose.

Medical Editor

Andrew W Lawton, MD, Medical Director of Neuro-Ophthalmology Service, Section of Ophthalmology, Baptist Eye Center, Baptist Health Medical Center
Andrew W Lawton, MD is a member of the following medical societies: American Academy of Ophthalmology, Arkansas Medical Society, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Brian R Younge, MD, Professor of Ophthalmology, Mayo Clinic School of Medicine
Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.