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trigeminal neuralgia (tic douloureux)

Etiology: - compression of the trigeminal nerve a) between the gasserian ganglion & the pons b) vascular loop at the level of the root entry to the pons - usually the superior cerebellar artery c) benign tumors - meningioma - epidermoid cyst - acoustic neuroma d) nasopharyngeal carcinoma e) multiple sclerosis Epidemiology: 1) generally > 50 years of age 2) women affected more than men Clinical manifestations: 1) paroxysmal pain in the distribution of the trigeminal nerve a) quality: intense, stabbing, lacinating b) duration: a few seconds to 2 minutes c) frequency: 1-2 attacks/day to 10-20 attacks/hour d) diurnal variation: fewer episodes at night e) generally unilateral pain f) usually involves 2nd & 3rd divisions of the trigeminal nerve - cheeks, jaw, lips, gums, ear g) rarely involves the ophthalmic division h) pain may be spontaneous or triggered by sensory stimuli of the face or mouth - trigger points precipitate paroxysms - 24-49% of patients report continuous or long-lasting pain, burning, throbbing, or aching between paroxysmal pain [7] 2) examination of sensory & motor components of cranial nerve 5 is often normal 3) associated cranial nerve abnormalities suggest mass lesion Radiology: - contrast-enhanced magnetic resonance imaging (all patients) [6] - non-vascular structural pathology (15%) - brain tumor - multiple sclerosis - superior cerebellar artery involved in vascular compromise or structural compression of trigeminal nerve (85%) Differential diagnosis: 1) multiple sclerosis 2) postherpetic neuralgia 3) glossopharyngeal neuralgia 4) Raeder's paratrigeminal syndrome 5) atypical facial neuralgia 6) chronic cluster headache syndrome (Horton's cephalgia) 7) cluster headaches 8) post-traumatic facial neuralgia 9) temporomandibular joint (TMJ) syndrome 10) dental pain 11) sinus pain Management: 1) pharmacologic agents a) carbamazepine (Tegretol) is treatment of choice [2] - begin 200 mg/day - increase 100-200 mg every 2-3 days to a max of 1200 mg/day - divide larger doses TID - routine laboratory tests (periodic) - complete blood count (CBC) - liver function tests - 80% of patients obtain pain relief b) oxacarbazine is alternative to carbamazepine - 300-1800 mg/day [6] c) lamotrigine, gabapentin, botulinum toxin type A, pregabalin, baclofen, & phenytoin may be used either alone or as add-on therapy [6] d) intravenous fosphenytoin or lidocaine for acute pain [6] 2) surgery reserved for patients refractory to medical therapy a) at least 3 drugs or drug combinations should be tried before surgery is considered [2] b) gamma knife radiosurgery [2,3] c) percutaneous trigeminal neurolysis - placement of a needle or electrode through the foramen ovale to the trigeminal cistern of Meckel's cave - destruction of the gasserian ganglion & retrogasserian rootlets - glycerol injection - radiofrequency thermocoagulation (rhizotomy) - no general anesthesia d) microvascular decompression - general anesthesia - retromastoid craniectomy - removes offending vascular structure from nerve - spares trigeminal nerve

Related

trigeminal (semilunar) ganglion; gasserian ganglion trigeminal autonomic cephalgia trigeminal nerve (CN V)

General

cranial nerve disease (cranial neuropathy) neuralgia sign/symptom

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 1043-45
  2. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19 American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2021.
  3. 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. PMID: 17174762
  4. Bigal ME Diagnostic evaluation and treatment of trigeminal neuralgia. Curr Pain Headache Rep. 2009 Aug;13(4):256-7. PMID: 19586587
  5. Maarbjerg S, Gozalov A, Olesen J, Bendtsen L. Trigeminal neuralgia--a prospective systematic study of clinical characteristics in 158 patients. Headache. 2014 Nov-Dec;54(10):1574-82. Epub 2014 Sep 18. PMID: 25231219
  6. European Academy of Neurology Trigeminal Neuralgia Clinical Practice Guidelines (2019) Medscape - Aug 01, 2019. https://reference.medscape.com/viewarticle/916210
  7. Rothaus C Trigeminal Neuralgia NEJM Resident 360. August 19, 2020 https://resident360.nejm.org/clinical-pearls/trigeminal-neuralgia
  8. Gronseth G, Cruccu G, Alksne J 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. PMID: 18716236 Review.
  9. Krafft RM. Trigeminal neuralgia. Am Fam Physician. 2008 May 1;77(9):1291-6. PMID: 18540495 Free article. Review.
  10. Oomens MA, Forouzanfar T. Pharmaceutical Management of Trigeminal Neuralgia in the Elderly. Drugs Aging. 2015 Sep;32(9):717-26. PMID: 26336972 PMCID: PMC4579266 Free PMC article. Review.
  11. NINDS Trigeminal Neuralgia Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Trigeminal-Neuralgia-Information-Page