Trigeminal Neuralgia

Aka: Trigeminal Neuralgia, Tic Douloureux, Fothergill’s Disease

advertisement

II. History

  1. John Fothergill first accurately described Trigeminal Neuralgia in 1773
    1. Pearce (2003) J Neurol Neurosurg Psychiatry 74:1688 [PubMed]

III. Epidemiology

  1. Incidence: 15,000 new cases per year in U.S. (3.4 women or 5.9 men per 100,000 per year)
    1. Incidence in UK as high as 26.8 per 100,000, and 12.6 per 100,000 in Netherlands (but criteria vary)
    2. Primary care physicians may see a few cases during their entire practice career
    3. Incidence in Multiple Sclerosis patients: 1-2%
  2. Onset after age 40 years in 90% of cases
    1. Mean age of onset 50 years old, with a peak at age 60 to 70 years
    2. Incidence increases to 45.2 per 100,000 in men over age 80 years old
  3. More common in women by ratio of 2:1
  4. Familial association in 1-2% of Trigeminal Neuralgia patients

IV. Risk Factors

  1. Cerebrovascular Accident
  2. Hypertension in women

V. Causes

  1. Idiopathic
  2. Space occupying lesions
  3. Cerebral Arteriovenous Malformation
  4. Multiple Sclerosis (present in 2-4% of Trigeminal Neuralgia cases, RR 20)
    1. Consider in younger patients (20-30 years old), especially with other neurologic involvement
    2. Outside of Multiple Sclerosis, Trigeminal Neuralgia is typically a condition of older patients (see above)

VI. Pathophysiology

  1. Most often primary TN (classic) or idiopathic, but may be secondary to other causes (e.g. MS, CVA)
  2. Related to Trigeminal Nerve demyelination
    1. Demyelination due to compression from local structures (esp. Superior Cerebellar Artery)
      1. Narrow foramen ovale may also contribute
    2. Demyelinated fibers are hyperactive, sending ectopic impulses with altered transmission
    3. Demyelinated fibers are more prone to ephaptic conduction
      1. Light touch impulses transmit to nearby pain fibers
  3. Most common site at cerebellopontine nerve root area
  4. Effects all branches of the Trigeminal Nerve (Right side is more commonly involved)
    1. Maxillary branch is most commonly involved
    2. Ophthalmic branch is least commonly involved

VII. Symptoms

  1. Facial pain in Trigeminal Nerve distribution
    1. Recurrent paroxysms of sharp, stabbing or lancinating pain
  2. Distribution
    1. Maxillary and mandibular branches of the Trigeminal Nerve (CN 5) are most commonly affected
    2. Each attack is unilateral (may alternate sides in up to 3-5% of cases)
  3. Characteristics
    1. Lancinating or stabbing pain that is severe and intense
    2. Electric shock type pain
    3. Facial spasms related to paroxysms of pain (Tic Douloureux) may occur
  4. Timing
    1. Each attack lasts for seconds to minutes
    2. Attacks may occur as often as multiple times daily (as many as 100/day) or as infrequently as monthly
    3. Attacks become more frequent and severe over time (and more refractory to medication)
    4. Attacks are rare during sleep
    5. Remissions of more than 6 months occur in 50% of patients
  5. Triggers
    1. Washing face
    2. Tooth Brushing
    3. Cold exposure
    4. Chewing
    5. Trigger Zones (pathognomonic for Trigeminal Neuralgia)
      1. Small areas in the region of the nose and mouth
      2. Light touch or other minimal stimulation in these zones triggers an attack
  6. Associated Findings
    1. Autonomic findings (Lacrimation, Eye Redness) may occur with paroxysmal pain episodes

VIII. History: Red Flags suggesting secondary cause or alternative diagnosis

  1. Abnormal findings on Neurologic Examination (e.g. intracranial lesion)
  2. Abnormal findings on examination of head and neck (e.g. dental or ear-related source)
  3. Age under 40 years old
  4. Severe, lancinating paroxysmal pain episode lasts longer than 2 minutes
  5. Pain outside the Trigeminal Nerve distribution
  6. Bilateral pain during a single attack
    1. Different attacks may affect other side in 3% of patients
  7. Vision change, Hearing change or Vertigo
  8. Numbness
  9. Findings suggestive of Multiple Sclerosis (e.g. Ataxia, unilateral Vision change)
    1. Multiple Sclerosis is often comorbid with Trigeminal Neuralgia

IX. Examination

  1. Evaluate for focal findings suggestive of a secondary cause or alternative diagnosis
  2. Specific focal areas of examination (abnormalities suggest alternative diagnosis)
    1. Temporomandibular Joint
    2. Facial Muscle Strength and symmetry
    3. Corneal Reflex
    4. Trigeminal NerveSensation (normal in Trigeminal Neuralgia)
      1. Trigger Zone presence is pathognomonic for Trigeminal Neuralgia (see above)

X. Diagnosis: Classic Trigeminal Neuralgia (ICHD3 Criteria)

  1. Recurrent paroxysms of unilateral facial pain
    1. Follows distribution of one or more Trigeminal Nerve and no radiation beyond
  2. Pain meets all 3 criteria
    1. Painful paroxysms last <=2 minutes
    2. Severe intensity
    3. Electric shock-like shooting, stabbing or sharp pain
  3. Triggered by innocuous stimuli in the affected trigeminal distribution (e.g. Trigger Zones)
  4. Not better accounted for by another ICHD-3 Diagnosis

XI. Types: Trigeminal Neuralgia (ICHD3 Categories)

  1. Classic (purely paroxysmal)
    1. Recurrent attacks of unilateral facial pain meeting classic Trigeminal Neuralgia criteria (as above)
    2. Pain-free in the affected trigeminal region between attacks
  2. Classic with concomitant persistent facial pain
    1. Recurrent attacks of unilateral facial pain meeting Trigeminal Neuralgia criteria (as above)
    2. Continuous or near continuous pain in the affected trigeminal region between attacks
      1. Aching, lower level pain persists between episodes
  3. Secondary Trigeminal Neuralgia
    1. Recurrent attacks of unilateral facial pain meeting Trigeminal Neuralgia criteria (paroxysmal or persistent)
    2. Underlying causative condition identified
    3. Not better attributed to another disorder
  4. Idiopathic Trigeminal Neuralgia
    1. Recurrent attacks of unilateral facial pain meeting Trigeminal Neuralgia criteria (paroxysmal or persistent)
    2. Does not meet criteria for classic or for secondary after thorough investigation (e.g. Brain MRI, EMG)
    3. Not better attributed to another disorder

XII. Differential Diagnosis

  1. Cluster Headache or other Migraine Headache
  2. Postherpetic Neuralgia
  3. Glossopharyngeal Neuralgia
  4. Dental Infection or Dental Caries
  5. Temporomandibular Joint Syndrome
  6. Acoustic Neuroma
  7. Multiple Sclerosis (may be comorbid)
  8. Vascular Malformation

XIII. Labs

  1. No specific lab testing is indicated for Trigeminal Neuralgia (unless exploring differential diagnosis)

XIV. Imaging

  1. Brain MRI (with and without contrast) Indications
    1. Indicated in most cases of Trigeminal Neuralgia at onset
    2. Intracranial lesions are present in up to 10% of cases
    3. Evaluate for other diagnoses (e.g. MS, Cerebral Aneurysm)
    4. Identify surgical intervention opportunities (e.g. narrow foramen ovale)
  2. Brain MRA and Neck MRA
    1. Consider for evaluation of neurovascular compression and other findings (e.g. AV Malformation)

XV. Diagnostics

  1. Trigeminal reflex testing (via EMG testing)
    1. Indicated in atypical presentations
    2. Differentiates classic from secondary Trigeminal Neuralgia with high efficacy
    3. Cruccu (2006) Neurology 66:139-41 [PubMed]

XVI. Management: General

  1. Neurology referral
    1. Evaluate for comorbid conditions (Multiple Sclerosis, Intracranial Lesions)

XVII. Management: Antiepileptics (and othe neurologic agents)

  1. Precautions
    1. Agents are initially effective in 75% of patients
    2. Efficacy wanes with time and symptoms may then worsen and become refractory
  2. Mechanism
    1. Seizure medications stabilize Neuron membrane and decrease excitability and erratic pain signal transmission
  3. Carbamazepine (Most studied)
    1. Dosing
      1. Start 200 mg orally twice daily
      2. Titrate as needed to effect over the course of weeks to 800 mg/day (typical effective dose)
      3. Maximum daily dose: 1200 mg/day for Trigeminal Neuralgia
    2. Efficacy
      1. Initial excellent response rates >70% (NNT 2)
      2. Longterm failure rate approches 50% after 5-10 years of continuous use
  4. Oxcarbazepine (Trileptal)
    1. Dosing
      1. Initial: 300 mg orally twice daily
      2. Increase by 300 mg/day every 3 to 7 days
      3. Typical effective dose 1200 mg/day divided twice daily
      4. Maximum: 1800 mg/day for Trigeminal Neuralgia
    2. Efficacy
      1. Effective for pain reduction
      2. Fewer side effects than Carbamazepine, but less effective in the longterm
  5. Baclofen (Lioresal)
    1. Typical effective doses: 10-80 mg/day
    2. Consider in Multiple Sclerosis patients with Trigeminal Neuralgia
  6. Agents with unknown effectiveness (inadequate studies as of 2025)
    1. Phenytoin (Dilantin)
    2. Gabapentin (Neurontin)
    3. Topiramate (Topamax)
    4. Sumatriptan (Imitrex)
    5. Lamotrigine (Lamictal)
      1. May worsen MS symptoms
  7. References
    1. Delzell (1999) Arch Fam Med 8(3): 264-8 [PubMed]

XVIII. Management: Other Symptomatic Therapy

  1. Topical Capsaicin
  2. Intranasal Lidocaine 8%
    1. Indicated for rescue therapy of acute Trigeminal Neuralgia exacerbations
    2. Sprayed on the nasal and/or Oral Mucosa
    3. Blocks the sphenopalatine Ganglion (for second Trigeminal Nerve branch)
    4. Zhou (2023) Cephalalgia 43(5):3331024231168086 +PMID: 37032614 [PubMed]
  3. Onabotulinumtoxin A (Botox)
    1. May decrease intensity and frequency of attacks at 6 weeks to 3 months
    2. Rubis (2020) J Oral Maxillofac Res 11(2): e2 [PubMed]
  4. Transcutaneous electrical nerve stimulation (TENS)
    1. May decrease pain during flares
    2. Motwani (2023) J Clin Exp Dent 15(6): e505-10 [PubMed]
  5. Acupuncture is ineffective in Trigeminal Neuralgia
    1. Millan-Guerrero (2006) Headache 46(3): 532 [PubMed]

XIX. Management: Surgical Management

  1. Indications for neurosurgery referral
    1. Refractory Trigeminal Neuralgia (or when medications are poorly tolerated)
    2. Secondary Trigeminal Neuralgia
    3. Structural pathology (e.g. narrow foramen ovale) on imaging
  2. Percutaneous Methods (non-invasive but short lasting)
    1. Glycerol injection
    2. Gamma Knife Stereotactic Radiosurgery
    3. Radiofrequency thermocoagulation or rhizotomy
      1. Effective, but risk of facial numbness and Corneal insensitivity
    4. Oturai (1996) Clin J Pain 12(4):311-5 [PubMed]
  3. Invasive Surgical Techniques
    1. Microvascular decompression (posterior fossa exploration)
      1. Most effective and long lasting (duration of 10 years in 70% of cases)
      2. Preferred over sterotactic radiosurgery
      3. Risk of unilateral Hearing Loss in 5% of cases
      4. Hai (2006) Neurol India 54(1):53-6 [PubMed]
      5. Tronnier (2001) Neurosurgery 48(6): 1261-8 [PubMed]
    2. Peripheral Nerve field stimulation
      1. Implanted device near Peripheral Nerves to disrupt pain signals
      2. Up to 75% pain improvement in observational studies
      3. Sarica (2022) J Neurosurg 137(5): 1387-95 [PubMed]

XX. Complications

  1. Major Depression and Suicidality (due to severity of pain and incapacity)

XXI. Course

  1. Remission is typical for months-years (>=6 months in >50% of patients)
  2. Multiple Sclerosis is associated with a more refractory course (to medications, interventions)

XXII. References

  1. Amaechi (2025) Am Fam Physician 111(5): 427-32 [PubMed]
  2. Krafft (2008) Am Fam Physician 77(9):1291-6 [PubMed]
  3. Kumar (1998) Postgrad Med 104(4):149-56 [PubMed]
  4. Scrivani (2005) Oral Surg Oral Med Oral Pathol Oral Radiol Endod 100(5):527-38 [PubMed]
  5. Zakrzewska (2016) Am Fam Physician 94(2): 133-5 [PubMed]

Images: Related links to external sites (from Bing)

These images are a random sampling from a Bing search on the term "Trigeminal Neuralgia." Click on the image (or right click) to open the source website in a new browser window. Search Bing for all related images

Related Topics in Headache Disorders

advertisement

OSZAR »