Trigeminal neuralgia ( TN or TGN), also called Fothergill disease, tic douloureux, or trifacial neuralgia, is a chronic pain disorder that affects the trigeminal nerve,[
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The typical form results in episodes of severe, sudden, shock-like pain in one side of the face that lasts for seconds to a few minutes. Groups of these episodes can occur over a few hours. The atypical form results in a constant burning pain that is less severe. Episodes may be triggered by any touch to the face.[ Both forms may occur in the same person. Pain from the disease has been linked to mental-health issues, especially depression.] (2025). 086715439X, Quintessence Publishing Co, Inc. 086715439X
The exact cause is unknown, but believed to involve loss of the myelin of the trigeminal nerve.
Treatment includes medication or surgery. The anticonvulsant carbamazepine or oxcarbazepine is usually the initial treatment, and is effective in about 90% of people.[ Opioids are not usually effective in the typical form. In those who do not improve or become resistant to other measures, a number of types of surgery may be tried.][
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Trigeminal neuralgia affects an estimated 0.03% to 0.30% of people around the world, with a 3:1 ratio between women and men.[ The condition was first described in detail in 1773 by John Fothergill.]
Signs and symptoms
This disorder is characterized by episodes of severe facial pain along the trigeminal nerve divisions. The trigeminal nerve is a paired cranial nerve that has three major branches - the ophthalmic nerve (V1), the maxillary nerve (V2), and the mandibular nerve (V3). Any or all branches of the nerve may be affected. Trigeminal neuralgia most commonly involves the middle branch (the maxillary nerve or V2) and lower branch (mandibular nerve or V3) of the trigeminal nerve.
An individual attack usually lasts from a few seconds to several minutes or hours, but these can repeat for hours with very short intervals between attacks. In other instances, only four to 10 attacks are experienced daily. The episodes of intense pain may occur . To describe the pain sensation, people often describe a trigger area on the face so sensitive that touching or even air currents can trigger an episode, but in many people, the pain is generated spontaneously without any apparent stimulation.
It affects lifestyle, as it can be triggered by common activities such as eating, talking, shaving, and brushing teeth. The wind, chewing, and talking can aggravate the condition in many patients. The attacks are said, by those affected, to feel like stabbing , burning, sharp, pressing, crushing, exploding, or shooting pain that becomes intractable.
The pain also tends to occur in cycles with remissions lasting months or even years. Pain attacks are known to worsen in frequency or severity over time in some people. Pain may migrate to other branches over time, but in some people remains very stable.
Bilateral (occurring on both sides) trigeminal neuralgia is very rare except for trigeminal neuralgia caused by multiple sclerosis (MS). This normally indicates problems with both trigeminal nerves, since one nerve serves the left side of the face and the other serves the right side. Occasional reports of bilateral trigeminal neuralgia reflect successive episodes of unilateral (only one side) pain switching the side of the face rather than pain occurring simultaneously on both sides.
Rapid spreading of the pain, bilateral involvement, or simultaneous participation with other major nerve trunks (such as painful tic convulsive of nerves V and VII or occurrence of symptoms in the V and IX nerves) may suggest a systemic cause. Systemic causes could include MS or expanding cranial tumors. (2025). 086715439X, Quintessence Publishing Co, Inc. 086715439X
The severity of the pain makes washing the face, shaving, and performing good oral hygiene more difficult. The pain has a significant impact on activities of daily living, especially as those affected live in fear of when they are going to get their next attack of pain and how severe it will be. It can lead to severe depression and anxiety.
Not all people, though, have the symptoms described above; several variants of TN occur, one of which is atypical trigeminal neuralgia (trigeminal neuralgia, type 2 or trigeminal neuralgia with concomitant pain),
Trigeminal pain can also occur after an attack of herpes zoster. Postherpetic neuralgia has the same manifestations as in other parts of the body. Herpes zoster oticus typically presents with inability to move many facial muscles, pain in the ear, taste loss on the front of the tongue, dry eyes and mouth, and a vesicular rash. Less than 1% of varicella zoster infections involve the facial nerve and result in this occurring.
Trigeminal deafferentation pain (TDP), also termed anesthesia dolorosa, or colloquially as phantom face pain, is from unintentional damage to a trigeminal nerve following attempts to fix a nerve problem surgically. TDP is usually constant with a burning sensation and numbness and is very difficult to treat, as further surgeries are usually ineffective and possibly detrimental to the person.
Causes
The trigeminal nerve is a mixed cranial nerve responsible for sensory data such as tactition (pressure), thermoception (temperature), and nociception (pain) originating from the face above the jawline; it is also responsible for the motor function of the muscles of mastication (those involved in chewing but not facial expression).
Several theories exist to explain the possible causes of this pain syndrome. The nerve was once thought to be compressed in the opening from the inside to the outside of the skull; but leading research indicates that it is an enlarged or lengthened blood vessel – most commonly the superior cerebellar artery – compressing or throbbing against the microvasculature of the trigeminal nerve near its connection with the pons.
Short-term peripheral compression is often painless.[ Persistent compression results in local demyelination with no loss of axon potential continuity. Chronic nerve entrapment results in demyelination primarily, with progressive axonal degeneration subsequently.][ It is, "therefore widely accepted that trigeminal neuralgia is associated with demyelination of axons in the Gasserian ganglion, the dorsal root, or both."] (2025). 086715439X, Quintessence Publishing Co, Inc. 086715439X
Trigeminal neuralgia is found in 3–4% of people with MS, according to data from seven studies.
Postherpetic neuralgia, which occurs after shingles, may cause similar symptoms if the trigeminal nerve is damaged, called Ramsay Hunt syndrome type 2.
When no structural cause is apparent, the syndrome is called idiopathic TN.
Diagnosis
Trigeminal neuralgia is diagnosed by the result of neurological and physical tests, as well as the individual's medical history.
As with many conditions without clear physical or laboratory diagnoses, TN is often misdiagnosed, and other conditions are also frequently misdiagnosed as TN.
Temporomandibular disorder (TMD) can present similarly to TN; differentiating between these conditions can be difficult.
Management
Some evidence points towards the need to quickly treat and diagnose TN. The longer a patient has TN, reversing the neural pathways associated with the pain are thought to be more difficult.
Medical
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The anticonvulsant carbamazepine is the first-line treatment; second-line medications include baclofen, lamotrigine, oxcarbazepine, phenytoin, topiramate, gabapentin, and pregabalin. Uncontrolled trials have suggested that clonazepam and lidocaine may be effective.
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Antidepressant medications, such as amitriptyline, have shown good efficacy in treating TN, especially if combined with an anticonvulsant drug such as pregabalin.
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Some evidence indicates that duloxetine can also be used in some cases of neuropathic pain, especially in patients with major depressive disorder,
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Controversy exists around opiate use such as morphine and oxycodone for treatment of TN, with varying evidence on its effectiveness for neuropathic pain. Generally, opioids are considered ineffective against TN, thus should not be prescribed.
Surgical
Microvascular decompression provides freedom from pain in about 75% of patients presenting with drug-resistant TN.
Surgical procedures can be separated into nondestructive and destructive:
Nondestructive
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Microvascular decompression involves a small incision behind the ear and some bone removal from the area. An incision through the meninges is made to expose the nerve. Any vascular compressions of the nerve are carefully moved and a sponge-like pad is placed between the compression and nerve, stopping unwanted pulsation and allowing myelin sheath healing.
Destructive
All destructive procedures cause facial numbness after relief, as well as pain relief.
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Percutaneous techniques, which all involve a needle or catheter entering the face up to the origin where the nerve splits into three divisions and then damaging this area, purposely, produce numbness, but also stop pain signals. These techniques are proven effective,
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Balloon compression uses inflation of a balloon at this point, causing damage and stopping pain signals.
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Glycerol injection, deposition of corrosive liquid glycerol at this point, causes damage to the nerve to hinder pain signals.
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Radiofrequency thermocoagulation rhizotomy is the application of a heated needle to damage the nerve at this point.
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Stereotactic radiosurgery is a form of radiation therapy that focuses high-power energy on a small area of the body.
Support
Psychological and social support have been found to play a key role in the management of chronic illnesses and chronic pain conditions, such as TN. Chronic pain can cause constant frustration to individuals and those around them.
History
Trigeminal neuralgia was first described by physician John Fothergill and treated surgically by John Murray Carnochan, both of whom were graduates of the University of Edinburgh Medical School. Historically, TN has been called "suicide disease" due to studies by the pioneering forefather in neurosurgery Harvey Cushing involving 123 cases of TN between 1896 and 1912. In those studies, it produced intense pain, higher rates of suicidal ideation in patients with severe migraines, and links to higher rates of depression, anxiety, and sleep disorders.
Society and culture
Some individuals of note with TN include:
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Four-time British Prime Minister William Gladstone is believed to have had the disease.
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Entrepreneur and author Melissa Seymour was diagnosed with TN in 2009 and underwent microvascular decompression surgery in a well-documented case covered by magazines and newspapers, which helped to raise public awareness of the illness in Australia. Seymour was subsequently made a Patron of the Trigeminal Neuralgia Association of Australia.
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Salman Khan, an Indian film star, was diagnosed with TN in 2011. He underwent surgery in the US.
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All-Ireland winning Christy Toye was diagnosed with the condition in 2013. He spent five months in his bedroom at home, returned for the 2014 season, and lined out in another All-Ireland final with his team.
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Jim Fitzpatrick – former Member of Parliament for Poplar and Limehouse – disclosed he had TN before undergoing neurosurgery. He has openly discussed his condition at parliamentary meetings and is a prominent figure in the TNA UK charity.
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Jefferson Davis – President of the Confederate States of America
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Charles Sanders Peirce – American philosopher, scientist, and father of pragmatism
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Gloria Steinem – American feminist, journalist, and social and political activist
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Anneli van Rooyen, Afrikaans singer-songwriter popular during the 1980s and 1990s, was diagnosed with atypical Tn in 2004. During surgical therapy directed at alleviating the condition performed in 2007, Van Rooyen had permanent nerve damage, resulting in her near-complete retirement from performing.
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H.R., singer of hardcore punk band Bad Brains
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Aneeta Prem, a British author, human rights campaigner, magistrate, and the founder and president of Freedom Charity experienced bilateral TN beginning in 2010, with severe pain and resulting sleep deprivation. Her condition remained undiagnosed until 2017. MVD surgery to ameliorate the pain on the right-hand side was performed at UCHL in December 2019.
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Travis Barker, drummer of rock band Blink-182
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Karel Heřmánek, Czech actor
See also
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Cluster headache
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Microvascular decompression
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Nerve compression syndrome
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Nerve decompression
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Trigeminal trophic syndrome
External links
