Home 5 Conditions 5 Trigeminal Neuralgia

Neurovascular Compression · A Particular Interest

Trigeminal Neuralgia

Also known as ‘tic douloureux’ — agonising facial pain that is highly treatable in experienced hands.

In brief

Sudden, severe, electric-shock-like pain on one side of the face
Diagnosis is made chiefly from a careful history — expertise matters
Most patients respond initially to medication (carbamazepine)
When medication fails, surgery offers excellent, lasting relief
Mr Russell manages 100+ new patients a year and offers every treatment option

Also known as ‘tic douloureux’, this is a type of neuralgia (nerve pain) that is associated with agonising facial pain that makes it difficult to talk or eat. The pain may be severe enough to cause spasms of the facial muscles (i.e. tic). Additional extreme distress may be associated with the recurring fear that the pain may return at any time, with resultant reduced quality of life. Trigeminal neuralgia is uncommon. Internationally, around 4–13 people per 100,000 develop it each year (its incidence),1 while studies estimate that up to roughly 0.3% of people are affected at some point in life (its lifetime prevalence).² There is no dedicated Australian study, but applying these international rates to Australia’s population of about 27 million suggests that somewhere between 25,000 and 75,000 Australians live with the condition — the Trigeminal Neuralgia Association of Australia puts the figure at up to 75,000.³ It is more common in women, and becomes more frequent with age, most often beginning between 50 and 70 years. Much can be done to help those afflicted with trigeminal neuralgia. Trigeminal neuralgia is a diagnosis based chiefly upon talking with the patient and obtaining a detailed history, thus good outcomes are based upon expertise in diagnosis. Once correctly diagnosed, treatment options include medication and neurosurgical procedures such as a microvascular decompression (MVD), needle rhizotomy (e.g. glycerol) and stereotactic radiosurgery (SRS).

What are the symptoms of trigeminal neuralgia?

Brief episodes of pain lasting from less than a second to a couple of minutes.
Often the attacks come in waves, with episodes lasting days to weeks then settling down for a period before returning.
Pain is usually stabbing, shooting, sharp or “like electricity”.
Pain is triggered by light facial touch such as talking, eating, brushing teeth, wind, etc
Pain is located in the face, most often the cheek, lips or jaw, but may also involve the side of the nose, inside of the mouth, eye or forehead.
Pain is usually confined to one side of the face, but may very occasionally affect both sides.
Pain usually initially responds to carbamazepine (Tegretol) or oxcarbazepine (Trileptal).

Are there different kinds of trigeminal neuralgia?

Internationally, trigeminal neuralgia is now classified as classical (due to compression of the nerve by a blood vessel), secondary (due to another condition such as multiple sclerosis or a tumour), or idiopathic (no cause found).⁴Each type may be either purely paroxysmal, with brief attacks only, or accompanied by concomitant continuous pain — a constant background aching or burning between attacks, which occurs in roughly a quarter to a half of patients.⁴ A more detailed surgical classification (the Burchiel system) is also used:⁵

Trigeminal neuralgia type 1 (TN1): classical form where episodic lancinating pain predominates, also known as “typical TN”.
Trigeminal neuralgia type 2 (TN2): atypical form where pain is aching, throbbing or burning and constant for >50% of the time, also known as “atypical TN”. TN2 is further divided into TN2a, where the constant pain has evolved from a preceding history of TN1, and TN2b, where the constant pain has been present from the onset without an earlier episodic phase.⁶
Trigeminal neuropathic pain (TNP): pain related to unintentional injury to the nerve from facial trauma, ENT surgery, oral surgery, posterior fossa surgery or stroke.
Trigeminal deafferentation pain (TDP): pain in an area of facial numbness, related to intentional injury to the nerve such as rhizotomy, neurectomy, gangliolysis or other denervating procedure.
Symptomatic trigeminal neuralgia (STN): pain associated with multiple sclerosis.
Persistent idiopathic facial pain (PIFP), previously called atypical facial pain: a separate, poorly understood chronic facial pain that does not follow the trigeminal neuralgia pattern and is distinguished from it.

What are the parts (divisions) of the trigeminal nerve?

Hence the name, the trigeminal nerve has three parts or divisions, and is the fifth of twelve pairs of nerves that supply the face and head. The first (ophthalmic or V1) supplies sensation to the forehead, eye and nose. The second (maxillary or V2) supplies the cheek, side of the nose, upper lip / teeth / gums. The third (mandibular or V3) supplies the jaw, lower lip / teeth / gums and tongue. Pain most commonly occurs in the second and third distributions, but may involve any of these. If the first division is involved, it tends to include the second as well. If pain is limited to the first, often it is not trigeminal neuralgia.

The three divisions of the trigeminal nerve — ophthalmic (V1), maxillary (V2) and mandibular (V3).

What causes trigeminal neuralgia?

The trigeminal nerve is responsible for carrying sensation from the face to the brain. Patients with trigeminal neuralgia have abnormal ‘wiring’ of the nerve causing ‘cross-talk’ between the parts of the nerve that convey normal non-painful sensation with those that carry painful stimuli. This is called ‘ephaptic’ transmission. Often this is caused by a blood vessel (artery or vein) compressing and distorting the trigeminal nerve just as it exits the brainstem, and it is thought that ongoing pulsations from these vessels may be responsible for the pain. In some cases, no vessel is found, even during surgery. Occasionally it may be caused by multiple sclerosis or a brain tumour. Each accounts for only a small proportion of cases — in the order of 2–5% — with a nearby tumour such as a meningioma or acoustic neuroma being the usual structural cause.⁷

How is trigeminal neuralgia treated?

Almost everyone begins with medication — this is the universal first step, and most patients gain at least good initial relief. Occasionally the pain settles on its own, but it almost always returns, and when it does it is usually worse than before. Over time some medications may cease to work or require higher doses, and they can cause significant side effects (described in the next section). Botulinum toxin (Botox) injections are a further option that may help some patients when tablets alone are insufficient; they tend to work best for people who have specific trigger points on the face that can be targeted directly.⁸ If medication no longer controls the pain, or the side effects become intolerable, a neurosurgical procedure should be considered. It is recommended that every patient with trigeminal neuralgia sees a neurosurgeon early in their journey — not because an operation will necessarily be offered, but so that they understand the full range of treatment options available to them from the outset.

What medicines are used in treating trigeminal neuralgia?

Ordinary pain-relief medications such as paracetamol, codeine and other opioids do not generally work well for this condition. The medications that do help are typically anti-seizure (anticonvulsant) drugs — though it is worth stressing that trigeminal neuralgia is neither a seizure nor a form of epilepsy; these drugs work by calming the abnormal nerve signalling that produces the pain.

Carbamazepine (Tegretol) is the first-line treatment and has the strongest evidence of any drug for trigeminal neuralgia, with the great majority of patients gaining good relief when it is first started.^8,9 It is begun at a low dose and increased gradually until the pain settles. Common side effects include drowsiness, dizziness, unsteadiness and nausea, and less commonly a low blood-sodium level or a skin rash; rare but serious reactions affecting the blood cells, liver or skin can occur, so occasional blood tests are recommended. In people of Asian descent a blood test (HLA-B*1502) is advised beforehand to identify the small group at higher risk of a severe skin reaction.

Oxcarbazepine (Trileptal) is a closely related first-line alternative of similar effectiveness that is often better tolerated and has fewer drug interactions, though it is somewhat more likely to lower the blood-sodium level, which is checked with blood tests.⁸ It is, however, considerably more expensive than carbamazepine.

If the first-line drugs fail or are not tolerated, a number of second-line agents may be used, either on their own or added to a first-line drug — these include gabapentin (Neurontin), sodium valproate, pregabalin (Lyrica), lamotrigine (Lamictal) and baclofen.⁸

In a severe, resistant crisis — an acute exacerbation with uncontrollable pain — hospital admission may be required, and intravenous medications such as lidocaine, phenytoin (or fosphenytoin) and ketamine can be used to bring the pain under control.⁸

What neurosurgical treatments are available to treat trigeminal neuralgia?

Microvascular decompression (MVD)
Percutaneous needle rhizotomy (PNR)
Stereotactic radiosurgery (SRS)

Who is a candidate for these procedures?

Patients for whom medications are no longer working despite increasing doses or who find the side effects of these medications intolerable.

What is a Microvascular Decompression (MVD)?

Microvascular decompression (MVD) is the only treatment that directly addresses the underlying cause of trigeminal neuralgia — the blood vessel compressing the trigeminal nerve where it leaves the brainstem. It offers the greatest chance of long-lasting, medication-free relief and is the preferred surgical option for patients with neurovascular compression.

The operation is performed under general anaesthetic. A small opening is made in the skull behind the ear (a retrosigmoid craniotomy) and, using a surgical microscope, the trigeminal nerve is identified at the brainstem. The offending artery is gently moved away from the nerve and held in its new position with a small piece of Teflon and tissue glue — a technique known as transposition. Only rarely is a vein responsible; it is almost always an artery.

An MVD offers an approximately 95% chance of immediate and complete resolution of the pain, with the ability to come off all medication. Most recurrences, when they occur, happen within the first two years, after which the yearly risk becomes very low — under 1% per year. In large published series, around 70% of patients remained free of pain without any medication ten years after surgery.¹⁰

The likelihood of any significant complication is very low. The main risks are:

Double vision — an approximately 5% risk of temporary (and less than 1% permanent) weakness of the fourth (trochlear) nerve, causing double vision that is particularly noticeable when reading.
Facial numbness — a 1–2% chance.
Facial weakness — a less than 1% chance.
Reduced hearing on the operated side — a 1–3% chance.
Brainstem injury — a less than 1 in 1000 chance, but potentially serious, with severe neurological consequences such as paralysis, sensory disturbance, or difficulty swallowing or speaking.
Return to theatre for bleeding — a less than 1% chance.
Infection — a 1–2% chance, often delayed by several months.
Cerebrospinal fluid (CSF) leak — a 1–2% chance.
General anaesthetic risks — including clots in the legs or lungs (DVT/PE), heart attack, stroke, severe allergic reaction. Taking the operation as a whole, the risk of death is very low — in the order of 0.2% (about 1 in 500).

Patients spend the first night in the Intensive Care Unit (ICU) prior to being transferred to the ward. Typical inpatient stay is between four and six nights. Significant headache, nausea and vomiting are common in the first day or two though quickly settle. Someone will need to care for you at home for the first week after discharge.

You will need to review with your GP 7–10 days after surgery to check the wound and confirm your recovery is progressing well. Sutures are self-dissolving and should not be removed. Providing the trigeminal pain has resolved, begin weaning trigeminal medication after 24 hours. Most patients are able to stop all trigeminal medication within a few weeks. You will review with the surgeon six weeks after surgery. Most people resume normal daily activities within one to two weeks, although usually unable to work for at least six weeks, and sometimes longer.

Microvascular decompression (MVD) — procedure animation A short medical animation of how the operation works

What is a Percutaneous Needle Rhizotomy?

This is a day procedure and usually the patient goes home later that day or the following morning. It is less invasive than an MVD but more than SRS. It is typically performed under general anaesthesia, although it can also be done under sedation. A specially designed needle is guided using X-ray and CT navigation to where the trigeminal nerve exits the skull. The nerve is treated with a chemical (glycerol), heat (radiofrequency electrocoagulation, RFE) or a balloon compression. All of these procedures work by deliberately damaging the nerve — in essence, trading the pain for permanent facial numbness. As a rule, the greater the numbness produced, the longer the period of freedom from pain. Treatment is therefore usually begun with glycerol, which creates the least numbness and allows a person to become accustomed to a numb face before progressing, if needed, to techniques that produce greater numbness. Most patients (around 75–80%) gain significant or complete relief of their pain. This relief typically lasts between one and four years — sometimes shorter, sometimes longer — after which the procedure can be repeated.¹¹

Facial numbness is the major drawback to a rhizotomy, and some reduction in facial sensation is almost inevitable. Although unsettling at first, once a person has become accustomed to it — usually within one to two months — it is not normally too troubling. Other risks include weakness of chewing on the treated side (around 5%), double vision (less than 1%), a less than 1% risk of injury to the nearby carotid artery (which can rarely cause a stroke or an abnormal artery-to-vein connection needing further treatment), meningitis (less than 1%), the rare possibility of anaesthesia dolorosa (constant, difficult-to-treat pain despite a numb face), and the usual anaesthetic risks — clots in the legs or lungs (DVT/PE), heart attack, stroke, severe allergic reaction, and a remote risk of death.

Percutaneous needle rhizotomy is generally reserved for:

atypical trigeminal neuralgia
trigeminal neuralgia secondary to multiple sclerosis
patients unfit for an MVD
patients not wishing to undergo an MVD

What is stereotactic radiosurgery (SRS)?

Stereotactic radiosurgery uses a high, focused dose of radiation (typically 70–90 Gy), planned jointly by a neurosurgeon and a radiation oncologist and delivered to the trigeminal nerve in a single session. It is non-invasive and avoids an operation, though it requires either a mask fitted over the face or a lightweight frame fixed to the head with pins. It is particularly useful for patients who cannot stop blood-thinning medication or for whom the other procedures are considered too risky. Around 80% of patients gain meaningful relief, although complete freedom from pain without any medication is achieved in roughly half — broadly comparable with the percutaneous options.¹² The effect is not immediate: relief usually begins within a few weeks and may take up to two months to reach its maximum. The benefit typically lasts between 18 months and four years, and about a quarter of patients experience a recurrence, after which repeat treatment is sometimes, though not always, possible. The main side effect is facial numbness, usually delayed, in around 15–20% of patients (troublesome in about 10%). Anaesthesia dolorosa — numbness accompanied by persistent pain — is rare, affecting about 1%, and some patients feel mildly tired or nauseated for a short period.

References

Helpful links

Video presentations

Mr Russell has recorded several patient-education talks on trigeminal neuralgia, including presentations for the Trigeminal Neuralgia Association Australia (TNAA).

Trigeminal neuralgia — treatment options TNAA webinar with Mr Jeremy Russell (2023)

Understanding trigeminal neuralgia TNAA webinar with Mr Jeremy Russell (2022)

Trigeminal neuralgia — a specialist conversation With pain physician Dr Nick Christelis



Trigeminal Neuralgia — patient information sheet PDF · prepared by Mr Jeremy Russell

Download PDF

Content on this page is for education and information only and is not a substitute for advice from your doctor. Medically reviewed by Mr Jeremy Russell.