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- Disorder of the sensory nucleus of the trigeminal nerve (cranial nerve [CN] V) that produces episodic, paroxysmal, severe, lancinating facial pain lasting seconds to minutes in the distribution of ≥1 divisions of the nerve
- Often precipitated by stimulation of well-defined, ipsilateral trigger zones: Usually perioral, perinasal, and, occasionally, intraoral (e.g., by washing, shaving)
- System(s) affected: Nervous
- Synonym(s): Tic douloureux; Fothergill neuralgia; Trifacial neuralgia; prosopalgia
- Women: 5.9/100,000/yr
- Men: 3.4/100,000/yr
- >70 years of age: ~25.6/100,000/yr
- Predominant age:
- >50 years; incidence increases with age
- Rare <35 years of age (consider another primary disease; see “Etiology”).
- Predominant sex: Female > Male (~2:1)
Unusual during childhood
Teratogenicity limits medical therapy during the 1st and 2nd trimesters.
- Demyelination around the compression site seems to be the mechanism by which compression of nerves leads to symptoms.
- Demyelinated lesions may set up an ectopic impulse generation causing erratic responses: Hyperexcitability of damaged nerves and transmission of action potentials along adjacent, undamaged, unstimulated sensory fibers.
- Compression of trigeminal nerve by anomalous arteries or veins of posterior fossa, compressing trigeminal root
- Etiologic classification:
- Idiopathic (classic)
- Secondary: Cerebellopontine angle tumors (e.g., meningioma); tumors of CN V (e.g., neuroma, vascular malformations), trauma, demyelinating disease (e.g., multiple sclerosis [MS])
Commonly Associated Conditions
- Sjögren syndrome
- Rheumatoid arthritis
- Chronic meningitis
- Acute polyneuropathy
- Hemifacial spasm
- Charcot-Marie-Tooth neuropathy
- Glossopharyngeal neuralgia