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- A painful 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 per year
- Men: 3.4/100,000 per year
- >70 years of age: ~25.6/100,000 per year
- Predominant age:
- >50 years; incidence increases with age.
- Rare: <35 years of age (Consider another primary disease; see “Etiology and Pathophysiology.”)
- Predominant sex: female > male (~2:1)
Unusual during childhood
Teratogenicity limits therapy for 1st and 2nd trimesters.
Etiology and Pathophysiology
- 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, and 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