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Neurology
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Pain Medicine

Trigeminal Neuralgia

High EvidenceUpdated: 2025-12-24

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Red Flags

  • Bilateral symptoms (suggests Multiple Sclerosis)
  • Sensory loss or numbness (suggests secondary cause)
  • Young age less than 40 years (consider MS or tumour)
  • Progressive symptoms (space-occupying lesion)
  • Other cranial nerve involvement
Overview

Trigeminal Neuralgia

1. Clinical Overview

Summary

Trigeminal neuralgia (TN) is characterised by recurrent, severe, unilateral, shock-like facial pain in the distribution of one or more branches of the trigeminal nerve (CN V). It is considered one of the most severe pain conditions known to medicine, often described as "the suicide disease." Classical TN is caused by vascular compression of the trigeminal nerve root (usually the superior cerebellar artery), while secondary TN occurs due to underlying pathology such as multiple sclerosis or tumours. First-line treatment is carbamazepine; surgical options include microvascular decompression for refractory cases. [1,2]

Key Facts

  • Incidence: 4-13 per 100,000 per year; increases with age. [3]
  • Peak Age: 50-70 years.
  • Sex: Female greater than Male (1.5:1).
  • Distribution: V2/V3 most common (95%); V1 alone rare (less than 5%).
  • Cause: Vascular compression (80-90%), MS (2-4%), tumour (less than 1%).
  • First-Line Treatment: Carbamazepine (or oxcarbazepine).
  • Surgical Cure: Microvascular decompression (MVD) - 80-90% success rate.

Clinical Pearls

"Suicide Disease": Trigeminal neuralgia is one of the most painful conditions known. The severity of pain can lead to depression and, historically, suicide - hence the name.

Trigger Zones: Light touch triggers the pain (eating, talking, washing face, wind); paradoxically, firm pressure often does NOT trigger pain.

Refractory Period: After an attack, there is typically a refractory period of seconds to minutes where another attack cannot be triggered.

Red Flags for Secondary TN: Age less than 40, bilateral symptoms, sensory loss, V1 involvement, or progressive course should prompt MRI to exclude MS or tumour.


2. Epidemiology

Incidence and Demographics

  • Annual Incidence: 4-13 per 100,000 population.
  • Prevalence: 0.1-0.2% of the population.
  • Peak Age: 50-70 years (rare before 40 unless secondary cause).
  • Sex: Female greater than Male (approximately 1.5:1).
  • Right greater than Left: Slight right-sided predominance.

Classification (ICHD-3)

TypeDefinition
Classical TNCaused by neurovascular compression at root entry zone
Secondary TNCaused by underlying disease (MS, tumour, AVM)
Idiopathic TNNo cause identified on investigation

Distribution by Nerve Branch

BranchPercentageTerritory
V2 (Maxillary)35%Cheek, upper lip, nose, upper teeth
V3 (Mandibular)30%Lower lip, chin, jaw, lower teeth
V2 + V330%Combined
V1 (Ophthalmic)Less than 5%Forehead, eye (rare alone; consider alternative diagnosis)

Risk Factors

Risk FactorMechanism
Age greater than 50Arterial elongation, tortuous vessels
HypertensionArterial elongation and ectasia
Multiple SclerosisDemyelinating plaques at root entry zone
Female sexUnknown; hormonal factors postulated
Family historyRare familial cases reported

3. Pathophysiology

Step 1: Neurovascular Compression

  • Offending Vessel: Usually superior cerebellar artery (SCA); less commonly AICA.
  • Site: Root entry zone (REZ) of trigeminal nerve - where central myelin transitions to peripheral myelin.
  • Compression: Pulsatile arterial pressure on nerve root.

Step 2: Focal Demyelination

  • Chronic mechanical compression → focal demyelination at REZ.
  • Disruption of myelin sheath exposes axons.

Step 3: Ephaptic Transmission

  • Demyelinated axons develop abnormal connections (ephapses).
  • Light touch fibres (Aβ) cross-stimulate pain fibres (Aδ, C).
  • This explains why light touch triggers severe pain.

Step 4: Central Sensitisation

  • Repeated painful stimuli → central hyperexcitability.
  • Trigeminal nucleus becomes sensitised.
  • Lower threshold for pain generation.

Step 5: Clinical Manifestation

  • Paroxysmal, stereotyped pain attacks.
  • Trigger zone stimulation → immediate pain paroxysm.
  • Refractory period follows (neuronal hyperpolarisation).

Pathophysiology Diagram

        VASCULAR COMPRESSION
        (Superior Cerebellar Artery)
                   ↓
┌────────────────────────────────────────┐
│   ROOT ENTRY ZONE COMPRESSION          │
│   Central-peripheral myelin junction   │
└────────────────────────────────────────┘
                   ↓
         FOCAL DEMYELINATION
                   ↓
┌────────────────────────────────────────┐
│   EPHAPTIC TRANSMISSION                │
│   Aβ (touch) → Aδ/C (pain) cross-talk  │
└────────────────────────────────────────┘
                   ↓
         CENTRAL SENSITISATION
         (Trigeminal nucleus)
                   ↓
┌────────────────────────────────────────┐
│   PAROXYSMAL PAIN ATTACKS              │
│   Light touch → severe pain            │
└────────────────────────────────────────┘

Secondary TN Mechanisms

Multiple Sclerosis

  • Demyelinating plaque at trigeminal root or nucleus.
  • 2-4% of MS patients develop TN.
  • Often bilateral; younger age of onset.

Tumours

  • CPA tumours (acoustic neuroma, meningioma) compress nerve.
  • May cause progressive sensory loss.

4. Clinical Presentation

Pain Characteristics

FeatureDescription
QualityElectric shock-like, shooting, stabbing, lancinating
IntensityExtremely severe (often described as "worst pain imaginable")
DurationSeconds to 2 minutes per attack; clusters possible
FrequencyMultiple attacks per day; may have remission periods
LocationUnilateral; V2/V3 distribution most common
Refractory PeriodSeconds to minutes post-attack where pain cannot be triggered

Trigger Factors

TriggerMechanism
TalkingMovement of jaw and facial muscles
Eating/ChewingJaw movement, touch on oral mucosa
Washing faceLight touch on trigger zone
Brushing teethIntraoral or facial touch
Cold windTemperature and tactile stimulation
ShavingTouch on affected area
Applying makeupLight facial touch

Natural History

Red Flags - "The Don't Miss" Signs

  1. Age less than 40 → Consider MS or tumour; MRI essential.
  2. Bilateral symptoms → Strongly suggests MS.
  3. Sensory loss or numbness → Suggests structural lesion.
  4. V1 involvement alone → Rare in classical TN; investigate.
  5. Progressive symptoms → CPA tumour, MS.
  6. Other cranial nerve signs → Space-occupying lesion.
  7. No trigger zone → Atypical; consider alternative diagnosis.

Attacks occur in clusters lasting weeks to months.
Common presentation.
Remission periods between clusters (months to years early on).
Common presentation.
Remissions shorten over time; attacks become more frequent.
Common presentation.
Some patients develop continuous background pain (TN type 2).
Common presentation.
5. Clinical Examination

General Observation

  • Patient may guard face or avoid touching affected side.
  • May grimace or wince during attack (tic douloureux = painful tic).
  • May have poor oral hygiene (avoiding brushing).

Neurological Examination

Cranial Nerves (Focus on V and Neighbours)

NerveTestNormal FindingRed Flag
V (Trigeminal)Light touch, pinprick all divisionsIntact sensationSensory loss
V MotorJaw clench, deviationSymmetricalWeakness, deviation
Corneal reflexCotton wispBlink presentAbsent (V1/VII issue)
VII (Facial)Facial movementsSymmetricalWeakness
VIII (Acoustic)Hearing, Rinne/WeberNormalHearing loss (CPA tumour)

Key Points

  • Classical TN: Examination should be NORMAL.
  • Any sensory or motor deficit suggests secondary cause.
  • Test all three divisions of trigeminal nerve.

Trigger Zone Identification

  • Lightly stimulate face with cotton wool.
  • Affected patients often refuse examination of trigger zone.
  • Location helps confirm diagnosis but rarely needed.

6. Investigations

Imaging

MRI Brain (Essential)

IndicationRationale
All patientsRule out secondary causes
Classical TN suspectedConfirm neurovascular conflict
Red flags presentMS plaques, CPA tumour

MRI Sequences

SequencePurpose
CISS/FIESTAHigh-resolution for vessel-nerve relationship
T2-weightedDemyelinating plaques (MS)
ContrastTumours, enhancement
MRAVascular anatomy

Other Investigations

InvestigationIndication
Lumbar punctureIf MS suspected (oligoclonal bands)
Evoked potentialsMS screen (VEPs)
Blood testsGenerally not helpful; routine bloods before carbamazepine

Diagnostic Criteria (ICHD-3)

Classical Trigeminal Neuralgia

  • A. At least 3 attacks of unilateral facial pain.
  • B. Occurring in one or more divisions of trigeminal nerve.
  • C. Pain has at least 3 of 4:
    1. Paroxysmal, lasting seconds to 2 minutes.
    2. Severe intensity.
    3. Electric shock-like, shooting, stabbing.
    4. Precipitated by innocuous stimuli to affected side.
  • D. No clinically evident neurological deficit.
  • E. Not better accounted for by another diagnosis.

7. Management

Management Algorithm

           TRIGEMINAL NEURALGIA DIAGNOSED
                        ↓
┌─────────────────────────────────────────────┐
│              MRI BRAIN                      │
│  - Exclude secondary causes                 │
│  - Look for neurovascular compression       │
└─────────────────────────────────────────────┘
                        ↓
            ┌───────────┴───────────┐
            ↓                       ↓
    CLASSICAL TN              SECONDARY TN
    (No structural lesion)    (MS, tumour)
            ↓                       ↓
FIRST-LINE MEDICAL            TREAT UNDERLYING
TREATMENT                     CAUSE + Pain Rx
            ↓
┌─────────────────────────────────────────────┐
│         CARBAMAZEPINE (First-line)          │
│  - Start 100mg BD                           │
│  - Titrate slowly to 200-400mg BD           │
│  - Maximum 1600mg/day                       │
│  OR OXCARBAZEPINE (better tolerated)        │
└─────────────────────────────────────────────┘
                        ↓
           ┌────────────┴────────────┐
           ↓                         ↓
    GOOD RESPONSE             INADEQUATE RESPONSE
    Continue Rx               or SIDE EFFECTS
    ↓                              ↓
MAINTENANCE                   SECOND-LINE
Regular review                ↓
Consider gradual         ┌────┴────────────────┐
taper if remission       ↓                     ↓
                    ADD/SWITCH TO:        CONSIDER
                    - Gabapentin          SURGERY
                    - Pregabalin               ↓
                    - Lamotrigine        SURGICAL
                    - Baclofen           OPTIONS:
                                         - MVD
                                         - Gamma Knife
                                         - Balloon compression

Medical Management

First-Line: Carbamazepine [4]

AspectDetail
Starting dose100mg BD
TitrationIncrease by 100-200mg every 2 days
Maintenance200-400mg BD-TDS
Maximum1600mg/day
Efficacy70-80% initial response
MonitoringFBC, LFTs, Na at baseline, 2 weeks, 3 months, then annually

Side Effects

  • Common: Drowsiness, dizziness, nausea, ataxia.
  • Serious: Hyponatraemia (SIADH), aplastic anaemia (rare), Stevens-Johnson syndrome (rare).
  • HLA-B*1502 screening: Asian populations (risk of SJS).

Oxcarbazepine (Alternative First-Line)

  • Better tolerated than carbamazepine.
  • Start 150mg BD, titrate to 300-600mg BD.
  • Lower risk of SJS but more hyponatraemia.

Second-Line Medications

DrugDoseNotes
Gabapentin300mg TDS-1200mg TDSAdd-on or monotherapy
Pregabalin75-300mg BDSimilar to gabapentin
Lamotrigine25-400mg/daySlow titration; add-on
Baclofen10-30mg TDSAdd-on; muscle relaxant
Phenytoin200-400mg/dayOlder agent; more side effects

Surgical Management

Indications

  • Failure of medical therapy.
  • Intolerable side effects from medications.
  • Patient preference for definitive treatment.

Surgical Options

ProcedureTechniqueSuccessRecurrenceNotes
Microvascular Decompression (MVD)Craniotomy; Teflon pad between vessel and nerve80-90%20-30% at 10 yearsBest for young, fit patients
Gamma Knife RadiosurgeryFocused radiation to nerve root70-80%Higher than MVDNon-invasive; delayed effect
Percutaneous RhizotomyNeedle ablation (RF, glycerol, balloon)70-90%Higher; may need repeatFor elderly or unfit

Microvascular Decompression (Janetta Procedure)

  • Gold standard surgical treatment.
  • Retrosigmoid (posterior fossa) craniotomy.
  • Identify and separate offending vessel.
  • Place Teflon pad between vessel and nerve.
  • Mortality: less than 0.5%.
  • Complications: Hearing loss (1-3%), CSF leak, facial numbness.

8. Complications

Disease-Related Complications

ComplicationFeatures
Weight lossAvoidance of eating due to pain
DehydrationAvoidance of drinking
Poor oral hygieneUnable to brush teeth
Depression/AnxietyChronic severe pain
Social isolationAvoidance of talking, eating with others
Suicidal ideation"Suicide disease" historically

Treatment-Related Complications

Carbamazepine

ComplicationManagement
HyponatraemiaCommon; check Na regularly; reduce dose or switch
Aplastic anaemiaRare; FBC monitoring
Stevens-Johnson SyndromeRare; HLA-B*1502 screening in Asians
HepatotoxicityLFT monitoring
Drowsiness/AtaxiaDose reduction

Surgical Complications

SurgeryComplications
MVDHearing loss, facial numbness, CSF leak, stroke (rare)
Percutaneous proceduresFacial numbness (expected), corneal anaesthesia, masseter weakness
Gamma KnifeDelayed onset of numbness, recurrence

9. Prognosis and Outcomes

Natural History

  • Spontaneous remissions occur (months to years) early in disease.
  • Over time, remissions become shorter and attacks more frequent.
  • Untreated, condition is progressive.

Treatment Outcomes

TreatmentInitial Success5-Year Success
Carbamazepine70-80%50-60% (may become refractory)
MVD80-90%70-80%
Gamma Knife70-80%50-60%
Percutaneous procedures70-90%40-60%

Prognostic Factors

Favourable

  • Classical TN with clear neurovascular conflict.
  • Good response to carbamazepine.
  • Younger, fit patients (for MVD).
  • Short duration of symptoms.

Unfavourable

  • Secondary TN (MS, tumour).
  • Long disease duration before surgery.
  • Multiple previous procedures.
  • Continuous background pain (TN type 2).

10. Evidence and Guidelines

Key Guidelines

GuidelineOrganisationKey Recommendations
NICE CKSUKCarbamazepine first-line; urgent referral if red flags
AAN/EAN Guidelines (2019)USA/EuropeCarbamazepine/oxcarbazepine first-line; surgery for refractory
ICHD-3 CriteriaInternational Headache SocietyDiagnostic criteria

Landmark Studies

1. Carbamazepine Efficacy (Cochrane 2019) [4]

  • Question: Is carbamazepine effective for TN?
  • Result: Carbamazepine is effective; NNT approximately 1.7-1.8.
  • Impact: Confirmed as first-line treatment.
  • PMID: 31034599.

2. Barker et al. MVD Outcomes (1996)

  • Question: Long-term outcomes of MVD for TN?
  • N: 1,185 patients with 10-year follow-up.
  • Result: 70% pain-free at 10 years.
  • Impact: Established MVD as durable surgical option.
  • PMID: 8637212.

3. Gamma Knife vs MVD (Comparison Studies)

  • MVD provides more durable pain relief.
  • Gamma Knife preferred for elderly/unfit.
  • Both superior to percutaneous procedures for long-term control.

11. Patient and Layperson Explanation

What is Trigeminal Neuralgia?

Trigeminal neuralgia (TN) is a condition that causes sudden, severe, shock-like pain in the face. The pain follows the course of the trigeminal nerve, which carries sensation from your face to your brain. It usually affects one side of the face and can be triggered by everyday activities.

What Causes It?

The most common cause is a blood vessel pressing on the trigeminal nerve near the brain. This causes the nerve to misfire, sending pain signals when there should be none. Other causes include multiple sclerosis (MS) or, rarely, a tumour.

What Does the Pain Feel Like?

  • Extremely severe, electric shock-like pain.
  • Lasts a few seconds to a minute or two.
  • Usually on one side of the face (cheek, jaw, or lips).
  • Comes in attacks, sometimes many times a day.
  • Can have pain-free periods lasting weeks or months.

What Triggers the Pain?

  • Touching your face.
  • Eating, chewing, or drinking.
  • Talking or smiling.
  • Brushing teeth.
  • Cold wind on your face.
  • Washing your face.

How is it Diagnosed?

  • Your doctor will ask about your symptoms.
  • Neurological examination should be normal.
  • An MRI scan is usually done to check for other causes.

How is it Treated?

Medication (First-Line)

  • Carbamazepine (Tegretol) is the main treatment.
  • It reduces the nerve's tendency to fire.
  • Needs regular blood tests to monitor.
  • Other medications include oxcarbazepine, gabapentin, or pregabalin.

Surgery (If Medication Doesn't Work)

  • Microvascular Decompression (MVD): Brain surgery to separate the blood vessel from the nerve. High success rate.
  • Gamma Knife: Focused radiation treatment. Non-invasive.
  • Needle Procedures: Ablation or compression of the nerve.

What is the Outlook?

  • Most people get good pain relief with medication or surgery.
  • Some people have remissions without treatment.
  • A small number have difficult-to-control pain.

When to Seek Help

  • If you have sudden, severe facial pain.
  • If treatments are not working.
  • If you develop numbness or weakness in your face.
  • If you are under 40 (may need extra tests).

12. References

Primary Sources

  1. Cruccu G, et al. Trigeminal neuralgia. Nat Rev Dis Primers. 2016;2:16065. PMID: 27734003.
  2. Jones MR, et al. A Comprehensive Review of Trigeminal Neuralgia. Curr Pain Headache Rep. 2019;23:74. PMID: 31456242.
  3. Maarbjerg S, et al. Trigeminal neuralgia – A prospective systematic study of clinical characteristics in 158 patients. Headache. 2014;54:1574-1582. PMID: 25231219.
  4. Di Stefano G, et al. Therapy for Trigeminal Neuralgia. Cochrane Database Syst Rev. 2019;12:CD007312. PMID: 31034599.
  5. NICE Clinical Knowledge Summaries. Trigeminal Neuralgia. https://cks.nice.org.uk/topics/trigeminal-neuralgia/.

Medical Disclaimer: MedVellum content is for educational purposes and clinical reference. Clinical decisions should account for individual patient circumstances. Always consult appropriate specialists.

Last updated: 2025-12-24

At a Glance

EvidenceHigh
Last Updated2025-12-24

Red Flags

  • Bilateral symptoms (suggests Multiple Sclerosis)
  • Sensory loss or numbness (suggests secondary cause)
  • Young age less than 40 years (consider MS or tumour)
  • Progressive symptoms (space-occupying lesion)
  • Other cranial nerve involvement

Clinical Pearls

  • **"Suicide Disease"**: Trigeminal neuralgia is one of the most painful conditions known. The severity of pain can lead to depression and, historically, suicide - hence the name.
  • **Trigger Zones**: Light touch triggers the pain (eating, talking, washing face, wind); paradoxically, firm pressure often does NOT trigger pain.
  • **Refractory Period**: After an attack, there is typically a refractory period of seconds to minutes where another attack cannot be triggered.
  • **Red Flags for Secondary TN**: Age less than 40, bilateral symptoms, sensory loss, V1 involvement, or progressive course should prompt MRI to exclude MS or tumour.

Guidelines

  • NICE Guidelines
  • BTS Guidelines
  • RCUK Guidelines