Condition
Trigeminal Neuralgia
Editors: Manjit S. Matharu MBChB, PhD, FRCP; Jennifer S. Kriegler MD; Esther Jolanda van Zuuren MD; Alexander Rae-Grant MD, FRCPC, FAAN
Produced in collaboration with American College of PhysiciansBackground Information
Description
Also Called
- Tic douloureux
- Trigeminal neuropathy
- Primary trigeminal neuralgia
Types
- International Headache Society classification of trigeminal neuralgia:
- Classical trigeminal neuralgia is categorized by magnetic resonance imaging (MRI) or surgery demonstrating neurovascular compression with morphological changes in trigeminal nerve root.
- Classical trigeminal neuralgia usually appears in the second or third divisions.
- It may be preceded by a period of atypical continuous pain (pre-trigeminal neuralgia).
- Subtypes:
- Classical trigeminal neuralgia, purely paroxysmal is when the patient is pain-free between attacks in the affected trigeminal distribution.
- Classical trigeminal neuralgia with concomitant continuous pain (previously called atypical trigeminal neuralgia, or type 2) is when the patient has continuous or near-continuous pain between attacks in the affected trigeminal distribution, which may be attributed to peripheral or central sensitization.
- Secondary trigeminal neuralgia (also called symptomatic trigeminal neuralgia) is caused by an underlying disease known to be able to cause neuralgia.
- Patients with secondary trigeminal neuralgia often exhibit sensory changes on clinical exam.
- Subtypes include:
- Trigeminal neuralgia attributed to multiple sclerosis
- Trigeminal neuralgia attributed to contact between the space-occupying lesion and the affected trigeminal nerve
- Trigeminal neuralgia attributed to a cause other than multiple sclerosis or a space-occupying lesion (such as a skull-base bone deformity, connective tissue disease, arteriovenous malformation, dural arteriovenous fistula, or a genetic cause)
- Idiopathic trigeminal neuralgia is a trigeminal neuralgia with no identifiable abnormalities upon electrophysiological testing or MRI.
- Findings may include contact between blood vessels and the trigeminal nerve and/or nerve root, but without evidence of morphological changes.
- Subtypes:
- Idiopathic trigeminal neuralgia, purely paroxysmal is when the patient is pain-free between attacks in the affected trigeminal distribution.
- Idiopathic trigeminal neuralgia with concomitant continuous pain is when the patient has continuous or near-continuous pain between attacks in the affected trigeminal distribution, which may be attributed to peripheral or central sensitization.
- Classical trigeminal neuralgia is categorized by magnetic resonance imaging (MRI) or surgery demonstrating neurovascular compression with morphological changes in trigeminal nerve root.
- Nervus intermedius neuralgia is a rare disorder (also known as geniculate neuralgia) which causes severe paroxysmal pain (lasting seconds to minutes) in the deep auditory canal, sometimes radiating to the parieto-occipital region.
- It is provoked by stimulating the posterior wall of auditory canal or periauricular region ("trigger area"). It is usually due to vascular compression of the nervus intermedius. The underlying cause may not be apparent, but it can be a complication of herpes zoster or, very rarely, multiple sclerosis or a tumor.
- PubMed28840083NMC case report journalNMC Case Rep J201706084375-7875Management of nervus intermedius neuralgia with microvascular decompression and/or surgical transection of the nervus intermedius has been reported in several case reports and case series.
- Reference - , (NMC Case Rep J 2017 Jul;4(3):75)
Epidemiology
Incidence/Prevalence
- Trigeminal neuralgia is rare, and commonly misdiagnosed or underdiagnosed.,
- It is slightly more common in women than men, and women typically have a younger age of onset.,
- The reported annual incidence of trigeminal neuralgia varies from 4-29 per 100,000 person-years (mean age of onset is 53-57 years) (Pract Neurol 2021 Oct;21(5):392).
- Estimated lifetime prevalence of trigeminal neuralgia is 0.16%-0.3% (Pract Neurol 2021 Oct;21(5):392).
- Patients with multiple sclerosis have a 1.9%-4.9% prevalence of secondary trigeminal neuralgia (J Headache Pain 2019 Feb 19;20(1):20).
Risk Factors
- There are no known risk factors for trigeminal neuralgia.
Associated Conditions
- Trigeminal neuralgia has a possible association with both hypertension and stroke.
Etiology and Pathogenesis
Causes
- Classical trigeminal neuralgia is caused by atrophy or displacement of the trigeminal nerve root due to neurovascular compression, typically by artery in cerebellopontine cistern.,
- Causes of secondary trigeminal neuralgia include:
- Multiple sclerosis plaques in pons or trigeminal root entry zone,
- Tumors (such as epidermoid tumor, meningioma, neurinoma),
- Arteriovenous malformation,
- Aneurysm
- Skull base bone deformity
- Connective tissue disease
- Dural arteriovenous fistula
- Genetic cause of neuropathy or nerve hyperexcitability
- Trigeminal neuralgia may also be idiopathic.,,,
- Tongue piercing was reported to cause trigeminal neuralgia in an 18-year-old woman in a case report (JAMA 2006 Oct 18;296(15):1840).
- Adjacent dental amalgams composed of dissimilar metals reported to cause trigeminal neuralgia in a 66-year-old woman in a case report (N Engl J Med 2000 Jun 29;342(26):2003).
- Herpes zoster-associated cranial nerve pain is considered to be a separate condition from trigeminal neuralgia (referred to as postherpetic neuralgia).
Pathogenesis
- Trigeminal neuralgia is typically caused by vascular compression of the trigeminal nerve at the nerve root entry zone, also known as a neurovascular conflict with compression.,
- This compression is hypothesized to result in central demyelination of the nerve root entry zone, leading to subsequent hyperexcitability of the nerve and impairment of the nociceptive system.,
- Demyelination may reach a level that allows ions to move more freely in and out of the axon on axonal segments other than the nodes of Ranvier, resulting in an inability to immediately re-establish resting potential.
- These axons then tend towards a state of depolarization, making them hyperexcitable.
- This hyperactivity of primary afferents may induce a central sensitization of wide-dynamic-range neurons in the spinal trigeminal nucleus.
- Crosstalk from nearby healthy nerve fibers may also contribute to hyperexcitability (ephaptic transmission).
- Patients with more background pain appear to have a loss of central inhibition of the nociceptive system.
- The transition zone from Schwann cell myelination to oligodendroglia myelination may represent an area particularly vulnerable to compression.
- In patients with trigeminal neuralgia due to multiple sclerosis, plaques can be found in the pons or trigeminal nerve root entry zone.
- If the plaques are located in the pons, this can affect the intrapontine central terminals of trigeminal afferents projecting to trigeminal brainstem nuclei.
- If the plaques are located in the nerve root entry zone, this can increase susceptibility of the nerve root to compression, increasing the likelihood of painful paroxysms.
Image 1 of 6
Trigeminal neurovascular conflict
Comparison of microsurgical anatomy, MRI-SPACE and MRA-TOF. Abbreviations: MRA, magnetic resonance angiography; MRI, magnetic resonance imaging; SPACE, Sampling Perfection with Application optimized Contrasts using different flip angle Evolution; TOF, Time of Flight; V, trigeminal nerve; VIII, vestibulocochlear nerve.
Produced in collaboration with American College of Physicians
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