Trigeminal neuralgia
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
Treatment algorithm
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
newly diagnosed trigeminal neuralgia (TN)
pharmacologic therapy
The mainstay of medical therapy consists of anticonvulsant medications, generally carbamazepine or oxcarbazepine. Carbamazepine is the only anticonvulsant medication with efficacy proven in randomized controlled trials is typically first-line therapy.[20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49.
https://onlinelibrary.wiley.com/doi/10.1111/ene.13950
http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com
[22]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.
https://n.neurology.org/content/71/15/1183
http://www.ncbi.nlm.nih.gov/pubmed/18716236?tool=bestpractice.com
[26]Yang F, Lin Q, Dong L, et al. Efficacy of 8 different drug treatments for patients with trigeminal neuralgia: a network meta-analysis. Clin J Pain. 2018 Jul;34(7):685-90.
http://www.ncbi.nlm.nih.gov/pubmed/29200017?tool=bestpractice.com
[27]Wiffen PJ, Derry S, Moore RA, et al. Carbamazepine for chronic neuropathic pain and fibromyalgia in adults. Cochrane Database Syst Rev. 2014 Apr 10;2014(4):CD005451.
http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD005451.pub3/full
http://www.ncbi.nlm.nih.gov/pubmed/24719027?tool=bestpractice.com
[ ]
How does carbamazepine affect outcomes in adults with chronic neuropathic pain?/cca.html?targetUrl=https://cochranelibrary.com/cca/doi/10.1002/cca.457/fullShow me the answer Almost 90% of patients achieve meaningful initial pain control with carbamazepine or oxcarbazepine.[4]Cruccu G, Di Stefano G, Truini A. Trigeminal neuralgia. N Engl J Med. 2020 Aug 20;383(8):754-62.
http://www.ncbi.nlm.nih.gov/pubmed/32813951?tool=bestpractice.com
However, long-term use may be associated with decreased efficacy.[2]Lambru G, Zakrzewska J, Matharu M. Trigeminal neuralgia: a practical guide. Pract Neurol. 2021 Oct;21(5):392-402.
https://pn.bmj.com/content/21/5/392
http://www.ncbi.nlm.nih.gov/pubmed/34108244?tool=bestpractice.com
Oxcarbazepine, a derivative of carbamazepine with fewer drug-drug interactions and a lower adverse effect profile, appears equally efficacious. The individual response to both drugs varies significantly; therefore, if one is not effective, the other can be trialled.[2]Lambru G, Zakrzewska J, Matharu M. Trigeminal neuralgia: a practical guide. Pract Neurol. 2021 Oct;21(5):392-402.
https://pn.bmj.com/content/21/5/392
http://www.ncbi.nlm.nih.gov/pubmed/34108244?tool=bestpractice.com
[3]Bendtsen L, Zakrzewska JM, Heinskou TB, et al. Advances in diagnosis, classification, pathophysiology, and management of trigeminal neuralgia. Lancet Neurol. 2020 Sep;19(9):784-96.
http://www.ncbi.nlm.nih.gov/pubmed/32822636?tool=bestpractice.com
If carbamazepine and oxcarbazepine are not tolerated or are ineffective, lamotrigine can be used as monotherapy or add-on therapy.[2]Lambru G, Zakrzewska J, Matharu M. Trigeminal neuralgia: a practical guide. Pract Neurol. 2021 Oct;21(5):392-402. https://pn.bmj.com/content/21/5/392 http://www.ncbi.nlm.nih.gov/pubmed/34108244?tool=bestpractice.com [3]Bendtsen L, Zakrzewska JM, Heinskou TB, et al. Advances in diagnosis, classification, pathophysiology, and management of trigeminal neuralgia. Lancet Neurol. 2020 Sep;19(9):784-96. http://www.ncbi.nlm.nih.gov/pubmed/32822636?tool=bestpractice.com [20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com One small randomized cross-over trial (n=14) showed that lamotrigine was efficacious as an add-on therapy for patients with refractory trigeminal neuralgia.[28]Zakrzewska JM, Chaudhry Z, Nurmikko TJ, et al. Lamotrigine (lamictal) in refractory trigeminal neuralgia: results from a double-blind placebo controlled crossover trial. Pain. 1997 Nov;73(2):223-30. http://www.ncbi.nlm.nih.gov/pubmed/9415509?tool=bestpractice.com
Gabapentin or pregabalin may also be used as monotherapy or add-on therapy.[2]Lambru G, Zakrzewska J, Matharu M. Trigeminal neuralgia: a practical guide. Pract Neurol. 2021 Oct;21(5):392-402. https://pn.bmj.com/content/21/5/392 http://www.ncbi.nlm.nih.gov/pubmed/34108244?tool=bestpractice.com [3]Bendtsen L, Zakrzewska JM, Heinskou TB, et al. Advances in diagnosis, classification, pathophysiology, and management of trigeminal neuralgia. Lancet Neurol. 2020 Sep;19(9):784-96. http://www.ncbi.nlm.nih.gov/pubmed/32822636?tool=bestpractice.com [4]Cruccu G, Di Stefano G, Truini A. Trigeminal neuralgia. N Engl J Med. 2020 Aug 20;383(8):754-62. http://www.ncbi.nlm.nih.gov/pubmed/32813951?tool=bestpractice.com [20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com Clinical experience indicates that these agents are less effective than carbamazepine or oxcarbazepine, but are associated with fewer adverse events.[4]Cruccu G, Di Stefano G, Truini A. Trigeminal neuralgia. N Engl J Med. 2020 Aug 20;383(8):754-62. http://www.ncbi.nlm.nih.gov/pubmed/32813951?tool=bestpractice.com
Development of allergic rash with use of carbamazepine or lamotrigine may be a precursor to the development of potentially serious reactions (e.g., Stevens-Johnson syndrome) and should prompt immediate discontinuation.
Patients whose TN is partially or completely refractory to anticonvulsant medications are often tried on other classes of pain medication, such as baclofen, although data supporting their use in TN are scant.[3]Bendtsen L, Zakrzewska JM, Heinskou TB, et al. Advances in diagnosis, classification, pathophysiology, and management of trigeminal neuralgia. Lancet Neurol. 2020 Sep;19(9):784-96. http://www.ncbi.nlm.nih.gov/pubmed/32822636?tool=bestpractice.com [20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com Baclofen may be helpful for TN in patients with multiple sclerosis who are using the drug for symptoms of spasticity.[2]Lambru G, Zakrzewska J, Matharu M. Trigeminal neuralgia: a practical guide. Pract Neurol. 2021 Oct;21(5):392-402. https://pn.bmj.com/content/21/5/392 http://www.ncbi.nlm.nih.gov/pubmed/34108244?tool=bestpractice.com Based on practitioner preference, it can be given sequentially as monotherapy or in combination until an effective regimen is established.
Primary options
carbamazepine: 200 mg/day orally initially given in 1-2 divided doses, usual maintenance dose is 400-1200 mg/day given in 2 divided doses
OR
oxcarbazepine: 300 mg/day orally initially, usual maintenance dose is 600-1200 mg/day, maximum 1200 mg/day given in 2 divided doses
Secondary options
gabapentin: 300 mg orally once daily initially, usual maintenance dose is 300-1800 mg/day, maximum 1800 mg/day given in 3 divided doses
OR
pregabalin: 75 mg orally (immediate-release) twice daily for 1 week, followed by 150 mg twice daily for 1 week, then 300 mg twice daily thereafter
OR
lamotrigine: consult specialist for guidance on dose
Tertiary options
baclofen: 10 mg orally three times daily initially, usual maintenance dose is 40-80 mg/day, maximum 80 mg/day
medication-unresponsive classical trigeminal neuralgia
microvascular decompression
First-line procedural treatment for classical TN, as it is targeted to the presumptive pathologic initiator of TN and has the least long-term neurologic sequelae, with rates and durability of symptom improvement that are equal to or better than other surgical/procedural treatments.[20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com [22]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. https://n.neurology.org/content/71/15/1183 http://www.ncbi.nlm.nih.gov/pubmed/18716236?tool=bestpractice.com [29]Laghmari M, El Ouahabi A, Arkha Y, et al. Are the destructive neurosurgical techniques as effective as microvascular decompression in the management of trigeminal neuralgia? Surg Neurol. 2007 Nov;68(5):505-12. http://www.ncbi.nlm.nih.gov/pubmed/17765958?tool=bestpractice.com A pooled analysis including 5149 patients showed that microvascular decompression is efficacious, with 62% to 89% of patients pain-free at follow-up (after 3-11 years). Severe complications, such as death, stroke, or meningitis, are rare. Other possible complications include cranial nerve palsy (4%), hearing loss (1.8%), and facial hypoesthesia (3%).[20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com
medication-unresponsive idiopathic trigeminal neuralgia
ablative surgery
There are a number of ablative techniques available.
Stereotactic radiosurgery involves targeting the sensory root of the trigeminal nerve stereotactically to deliver high doses of radiation without significant spread to surrounding tissues. It is the least invasive surgical option, and pain relief typically occurs within weeks to months with maximum pain improvement at 1 month. Between 30% and 66% of people undergoing stereotactic radiosurgery achieve long-term remission.[20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com The procedure should be carried out in specialist centers and patients should be selected by a multidisciplinary team experienced in the management of trigeminal neuralgia.[33]National Institute for Health and Care Excellence. Stereotactic radiosurgery for trigeminal neuralgia. Feb 2022 [internet publication]. https://www.nice.org.uk/guidance/ipg715
Trigeminal gangliolysis can be thermal (radiofrequency) or chemical (glycerol). Between 26% and 82% of patients achieve remission after radiofrequency gangliolysis and 19% to 58% achieve remission after glycerol injection.[20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com
Balloon compression of the trigeminal ganglion requires general anesthesia. It has similar efficacy and recurrence rates as gangliolysis but with decreased rates of severe dysesthesia and impaired corneal sensation. Pooled analyses reported that with a follow-up of 4-11 years, 55% to 80% of patients (n=755) with trigeminal neuralgia were pain-free after balloon compression.[20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com
The most common complications for ablative procedures are facial hypoesthesia (19%), corneal hypoesthesia (5%), and trigeminal motor weakness (5%). Meningitis (0.7%) and anesthesia dolorosa (0.5%) are rare complications.[20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com
medication-unresponsive secondary trigeminal neuralgia
individualized therapy
Patients with secondary TN generally respond less well to medical therapy or surgical treatment. As no treatment has sufficient evidence to prove its specific efficacy in patients with secondary TN, they should be treated similarly to patients with primary TN.[20]Bendtsen L, Zakrzewska JM, Abbott J, et al. European Academy of Neurology guideline on trigeminal neuralgia. Eur J Neurol. 2019 Jun;26(6):831-49. https://onlinelibrary.wiley.com/doi/10.1111/ene.13950 http://www.ncbi.nlm.nih.gov/pubmed/30860637?tool=bestpractice.com The choice of procedure is ultimately dependent upon patient preference but should only be undertaken after a complete discussion and understanding of the efficacy, recurrence rates, and potential complications of each method.
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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer
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