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Guidelines to Practice

Guidelines for the management of trigeminal neuralgia

Mun Seng Chong, MBBS, MD, FRCP, Anish Bahra, MBChB, MD, FRCP and Joanna M. Zakrzewska, BDS, MB BChir, MD, FDSRCS, FFDRCSI, FFPM RCA, FHEA
Cleveland Clinic Journal of Medicine June 2023, 90 (6) 355-362; DOI: https://doi.org/10.3949/ccjm.90a.22052
Mun Seng Chong
Consultant Neurologist, National Hospital for Neurology and Neurosurgery, London, UK; Member, Medical Advisory Committee for Trigeminal Neuralgia Association UK
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  • For correspondence: [email protected]
Anish Bahra
Consultant Neurologist, National Hospital for Neurology and Neurosurgery, London, UK; Cleveland Clinic London, UK
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Joanna M. Zakrzewska
Professor of Pain in Relation to Oral Medicine, National Hospital for Neurology and Neurosurgery, London, UK; Royal National ENT & Eastman Dental Hospitals, UCLH NHS Foundation Trust, London, UK; Cleveland Clinic London, UK; Co-Founder, Trigeminal Neuralgia Institute; Member of European and UK guidelines committee for trigeminal neuralgia; Principal Investigator for phase 2 drug trials for trigeminal neuralgia
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    TABLE 1

    Key symptoms of trigeminal neuralgia and differential diagnosis

    Key symptomsOther possible symptomsDifferential diagnosis
    Paroxysmal pain
    • Sharp and shooting

    • Lasts seconds to minutes

    • Provoked by light touch

    Burning, prickling, dull tender constant background painTrigeminal neuralgia with concomitant, continuous pain
    Trigeminal nerve innervation areaInterparoxysmal painTemporomandibular disorder
    Pain cannot be evoked between attacks (refractory period)Autonomic symptomsaSUNCT and SUNA
    Periods of remission or relapseSensory changebPainful trigeminal neuropathy
    Abrupt onsetAfter eatingDental, cracked tooth
    • ↵a Some facial reddening and tearing, sometimes on both sides, may be seen during acute pain paroxysms. If more pronounced with strictly unilateral conjunctival reddening, eyelid droop, nasal blockage, then consider SUNCT and SUNA.

    • ↵b During a relapse of trigeminal neuralgia and especially just after paroxysms of pain, there may be subtle transient unilateral sensory change in the area innervated by the trigeminal nerve. The presence of permanent sensory alterations and atypical features such as absent refractory period and no pain remission raise the possibility of trigeminal nerve damage and painful trigeminal neuropathy.

    • SUNA = short-lasting unilateral neuralgiform headache attacks with autonomic features; SUNCT = short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing

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    TABLE 2

    Classification of trigeminal neuralgia

    TypeSubtypes
    Classic trigeminal neuralgia (neurovascular compression present)
    • Purely paroxysmal

    • Concomitant continuous pain

    Secondary trigeminal neuralgia (underlying pathology present)
    • Attributed to multiple sclerosis

    • Attributed to space-occupying lesion

    • Atributed to other causes

    Idiopathic trigeminal neuralgia (no underlying cause found)
    • Purely paroxysmal

    • Concomitant continuous pain

    • Based on data from reference 4.

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    TABLE 3

    Evidence-based pharmacotherapy for trigeminal neuralgia

    DrugDosage regimenUsual dose rangeCommon side effects
    First-line therapy
     Carbamazepinea100 mg twice daily; double after 3 days to 200 mg twice daily; increase by 100–200 mg twice daily every 3 days, then 200 mg 4 times daily800–1,200 mg dailySedation, dizziness, blurred vision, nausea, unsteady lethargy, double vision, headache
    May cause hyponatremia, skin rashes, pancytopenia
    Risk of osteoporosis with long-term use
    May reduce oral contraception efficacy
     Oxcarbazepinea150 mg twice daily, double to 300 mg twice daily after 3 days; increase by 150–300 mg twice daily every 3 days, then 300 mg 4 times daily1,200–1,800 mg dailyDrowsiness, dizziness, diplopia, confusion, nausea, abdominal pain, headache, depression, diarrhea
    High risk of hyponatremia
    Chronic use risks osteoporosis
    May reduce oral contraception efficacy
    Second-line therapy
     LamotrigineStart 25 mg a day for 7 days, then 25 mg twice daily for 7 days, then 50 mg twice daily for another week; subsequent dose increments of 50 mg every 7 days, up to 10 weeks200 mg twice dailyBlurred vision, agitation, aggression, unsteadiness, dizziness, nausea, dry mouth, insomnia, joint pains
    Risk of skin rashes and Stevens-Johnson syndrome with rapid dose escalation
    Probably safe for pregnant women at a dose of 100 mg twice daily
     Baclofen5 mg 3 times daily for 3 days, then increase to 10 mg 3 times daily for 3 days; increase by 10 mg 3 times daily every 3 days until maximum dose40–80 mg dailyAnxiety, depression, agitation, unsteadiness, headache, sedation, tremor, skin rash, blurred vision, dry mouth, abdominal pain, withdrawal symptoms if stopped too rapidly
     Gabapentin100 mg 3 times daily day 1, 200 mg 3 times daily day 2 and 300 mg 3 times daily day 3. Increase by 1-300 mg 3 times daily every 3 days to maximum dose; start 100 mg 3 times daily or 300 mg at bedtime; can increase dose up to 300 to 600 mg 3 daily900–3,600 mg dailyAmnesia, confusion, dizziness, vertigo, drowsiness, depression, nausea, blurred vision, peripheral edema, constipation, abdominal bloating, weight gain
     Pregabalin25 mg twice daily; increase by 25–50 mg twice daily every 3 days600 mg dailyConfusion, drowsiness, constipation, blurred vision, dizziness, nausea, peripheral edema, increased appetite, weight gain
    • ↵a Carbamazepine and oxcarbazepine are available in liquid form. Dosage ranges vary due to lack of high-quality trials.

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    TABLE 4

    Treatments for acute episodes of trigeminal neuralgia based on a systematic review

    ProviderTreatment
    All clinicians (dentist, general practitioner, specialist)Lidocaine
    • 10-mg nasal spray, 2 sprays into nostril on affected side; can be used intraorally, but spit out after 1 minute

    • 5% ointment to trigger area

    • 2% 1:80,000 adrenaline local infiltration to nerve block trigger area

    General practitioner, specialistSumatriptan 6 mg subcutaneous injection, followed by oral sumatriptan 50 mg twice daily for 1 week
    Specialist onlyBotulinum toxin type A injection, 3 mg in 1 mL
    Specialist, inpatient basisIntravenous Infusions
    • Lidocaine 1.5 mg/kg over 1 hour, up to 5 mg/kg in a randomized clinical trial

    • Phenytoin 10 mg/kg

    • Fosphenytoin 15 mg/kg

    • Based on data from references 9 and 11.

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Cleveland Clinic Journal of Medicine: 90 (6)
Cleveland Clinic Journal of Medicine
Vol. 90, Issue 6
1 Jun 2023
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Guidelines for the management of trigeminal neuralgia
Mun Seng Chong, Anish Bahra, Joanna M. Zakrzewska
Cleveland Clinic Journal of Medicine Jun 2023, 90 (6) 355-362; DOI: 10.3949/ccjm.90a.22052

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Guidelines for the management of trigeminal neuralgia
Mun Seng Chong, Anish Bahra, Joanna M. Zakrzewska
Cleveland Clinic Journal of Medicine Jun 2023, 90 (6) 355-362; DOI: 10.3949/ccjm.90a.22052
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