The disorder is characterized by episodes of intense facial pain that last from a few seconds to several minutes or hours. Patients can pinpoint their pain accurately. The pain is not restricted to any division of the trigeminal nerve but generally runs along the line dividing either the mandibular and maxillary nerves or the mandibular and ophthalmic divisions of the nerve. Ophthalmic branch involvement is seen in less than 5% of patients [12].

The pain comes in paroxysms. The patient may describe the sensation of pain, in a particular trigger area on the face which is so sensitive that touching or even air currents can cause the pain. In some patients, the pain may start without any stimulation also. Generally, these trigger zones are seen near the nose or mouth [13]. Common day to day activities like eating, smiling, shaving, talking, brushing teeth can also trigger pain. Often a loud noise, wind, high pitched sounds can also worsen the condition in few cases.

Patients define pain as if they feel like stabbing, burning, pressing, electric shock like, crushing or shooting pain that cannot be traced. At the time of attack, patients may wince, grimace or make a nasty head movement so as to get away from pain, thereby generating an apparent movement or tic and hence the term tic douloureux.

The number of attacks can differ from less than 1 per day to around 12 or more per hour and sometimes even up to hundreds per day. In the beginning, lot of patients develop pain in one branch and after a couple of years the pain travels to other branches. Few patients complain of pain in index finger along with too [14].

• Pain

  In the beginning, lot of patients develop pain in one branch and after a couple of years the pain travels to other branches. Few patients complain of pain in index finger along with too. [symptoma.com]

  Compared with patients with pain history of ≤5 years, patients with pain history of >5 years experienced longer latency before pain relief (P = 0.027). [ncbi.nlm.nih.gov]

  […] complete pain relief — BNI grade 1, with a subsequent recurrence of pain). [medscape.com]

• Severe Pain

  Despite extensive therapies, severe pain persisted. She received proton beam radiation with significant improvement. However, she developed radiation necrosis and hydrocephalus. [ncbi.nlm.nih.gov]

  […] medication; and BNI V score, severe pain or no pain relief. [ro-journal.biomedcentral.com]

  Trigeminal neuralgia causes brief, intense, severe pain, usually on one side of the face or the jaw or near the eye. Trigeminal neuralgia is a type of neuropathicpain (pain caused by nerves). [jamanetwork.com]

  Trigeminal Neuralgia Brain Tingling in the cheek or jaw Dull aching in the teeth or jaw Flashes of severe pain Anxiety Blood vessel pressing against the trigeminal nerve Multiple sclerosis Tumor Overview Trigeminal neuralgia is a type of chronic nerve [nm.org]

• Agent Orange

  Medical history revealed peripheral neuropathy and prior exposure to Agent Orange. Diagnostic testing on teeth #19, #20, and #21 ruled out an odontogenic cause for the pain. [ncbi.nlm.nih.gov]

• Jaw Pain

  This jaw pain often is believed to be a dental problem, resulting in unnecessary tooth removal that does not cure the pain. Neurosurgery One's (formerly South Denver Neurosurgery) J. [southdenverneurosurgery.org]

  Many times, the condition is misdiagnosed as dental or jaw pain, and a correct diagnosis can take years to obtain. [nyulangone.org]

  Pain felt near the lips, eyes, nose, scalp, forehead, upper and lower jaw. Pain triggered by touch, sounds or everyday activities. Pain episodes last from a few seconds to a couple minutes. [neurosurgeonsofnewjersey.com]

• Erythema

  Differential Diagnosis Dental infection or cracked tooth (well localised to tooth, local swelling and erythema). TMJ pain (often bilateral, may radiate around ear neck, limited jaw opening). [emed.ie]

• Eye Pain

  You have eye pain, eye numbness, or sudden vision or hearing changes. You have sudden dizziness, or problems with movement, weakness, or numbness in your face. Care Agreement You have the right to help plan your care. [drugs.com]

  Oculofacial pain: corneal nerve damage leading to pain beyond the eye. Invest Ophthalmol Vis Sci. 2016;57(13):5285–7. https://doi.org/10.1167/iovs.16-20557. [jmedicalcasereports.biomedcentral.com]

  Dental pathologies, temporomandibular joint pain, eye pain (including glaucoma, orbital cellulitis, and trauma), facial trauma and bony fractures, tumor of the facial bones or the trigeminal nerve, giant cell arteritis, Tolosa-Hunt syndrome (idiopathic [neurosurgicalatlas.com]

• Photophobia

  Migraine (aura, severe unilateral, assoc GI upset, photophobia). Temporal arteritis (constant pain, jaw claudication, fever, wt loss, tender non pulsatile temporal arts). [emed.ie]

  […] often bilateral; jaw claudication Glossopharyngeal neuralgia Pain in tongue, mouth, or throat; brought on by swallowing, talking, or chewing Intracranial tumors May have other neurologic symptoms or signs Migraine Longer-lasting pain; associated with photophobia [aafp.org]

• Facial Pain

  Facial pain is a perplexing problem confronting all who practice the healing arts. The purpose of this article is to concentrate on one aspect of facial pain--trigeminal neuralgia. [ncbi.nlm.nih.gov]

  Facial pain There is usually a sharp, deep, electric like, stabbing type unilateral facial pain, which is most often on the right side of the face, commonly localised to the line dividing the mandibular and maxillary nerves or the line dividing the mandibular [medicaljoyworks.com]

  These are not a substitute for seeing your doctor if you have facial pain. The answers may help you decide to see your primary care doctor or a facial pain specialist. Try our free interactive online facial pain diagnosis tool. [ohsu.edu]

• Facial Numbness

  Patients who underwent 2-isocenter GKS had a higher rate of postoperative facial numbness, but only 9 cases reported bothersome facial numbness. Multibranch involvement was a risk factor for postoperative facial numbness. [ncbi.nlm.nih.gov]

• Unilateral Facial Pain

  Trigeminal neuralgia is a syndrome of paroxysmal excruciating, lancinating unilateral facial pain. [ncbi.nlm.nih.gov]

  Abstract Background: Trigeminal neuralgia is a syndrome of paroxysmal excruciating, lancinating unilateral facial pain. [doi.org]

• Facial Spasm

  Trigeminal neuralgia (tic douloureux) is a facial pain syndrome which often accompanies a facial spasm. The pain is sudden and unpredictable, lasting for several seconds. It is a disorder of the trigeminal nerve in the head. [aanem.org]

  […] from mouth to the angle of the jaw on the affected side in most cases Occurs either at rest or triggered by movements such as chewing, talking or touch (e.g., brushing teeth, washing face ) ; becomes worse with stimulation Facial spasms may occur Psychological [amboss.com]

  Trigeminal neuralgia is characterized by facial pain often accompanied by a brief facial spasm or tic. [emedicine.medscape.com]

• Cheek Pain

  […] them, there may be no pain pain wherever the trigeminal nerve and its branches may reach, including the forehead, eyes, lips, gums, teeth, jaw, and cheek pain in one side of the face, or, less frequently, both sides pain that is focused in one spot or [medicalnewstoday.com]

  It can relieve nerve pain by destroying the part of the nerve that causes pain and suppressing the pain signal to the brain. The surgeon passes a hollow needle through the cheek into the trigeminal nerve. [aans.org]

• Anhedonia

  Google Scholar | Crossref | Medline | ISI Marbach, J.J. and Lund, P. ( 1981 ) Depression, anhedonia and anxiety in temporomandibular joint and other facial pain syndromes. Pain 11: 73 - 84. [doi.org]

  Depression, anhedonia and anxiety in temporomandibular joint and other facial pain syndromes. Pain. 1981; 11 (1):73–84. [ PubMed ] [ Google Scholar ] 4. Love S, Coakham HB. Trigeminal neuralgia: pathology and pathogenesis. [ncbi.nlm.nih.gov]

• Suicidal Depression

  The condition can lead to irritability, severe anticipatory anxiety and depression, and life-threatening malnutrition. Suicidal depression is not uncommon. [emedicinehealth.com]

• Suicidal Ideation

  TN patients will often experience depression (including suicidal ideation) and anxiety, and withdraw from social activities. [centrastate.com]

• Neuralgia

  Convert to ICD-10-CM : 350.1 converts directly to: 2015/16 ICD-10-CM G50.0 Trigeminal neuralgia Approximate Synonyms Left trigeminal neuralgia Right trigeminal neuralgia Trigeminal neuralgia (nerve pain) Clinical Information A syndrome characterized by [icd9data.com]

  Typical Trigeminal Neuralgia C. Atypical Trigeminal Neuralgia D. Pre-Trigeminal Neuralgia E. Multiple-Sclerosis-Related Trigeminal Neuralgia F. Secondary or Tumor Related Trigeminal Neuralgia G. [web.archive.org]

  Most cases of trigeminal neuralgia are caused by compression of the trigeminal nerve root. Depending on the etiology, trigeminal neuralgia can be classified as classic trigeminal neuralgia or painful trigeminal neuropathy. [ncbi.nlm.nih.gov]

  Google Scholar | Crossref | Medline | ISI Zakrzewska, J.M. ( 2006 ) Insights: facts and stories behind trigeminal neuralgia. Gainesville, FL : Trigeminal Neuralgia Association. [doi.org]

• Trigeminal Neuralgia

  Typical Trigeminal Neuralgia C. Atypical Trigeminal Neuralgia D. Pre-Trigeminal Neuralgia E. Multiple-Sclerosis-Related Trigeminal Neuralgia F. Secondary or Tumor Related Trigeminal Neuralgia G. [web.archive.org]

  Google Scholar | Crossref | Medline | ISI Zakrzewska, J.M. ( 2006 ) Insights: facts and stories behind trigeminal neuralgia. Gainesville, FL : Trigeminal Neuralgia Association. [doi.org]

  Most cases of trigeminal neuralgia are caused by compression of the trigeminal nerve root. Depending on the etiology, trigeminal neuralgia can be classified as classic trigeminal neuralgia or painful trigeminal neuropathy. [ncbi.nlm.nih.gov]

  Idiopathic trigeminal neuralgia typically occurs in patients in the sixth decade of life, but it may occur at any age. Symptomatic or secondary trigeminal neuralgia tends to occur in younger patients. [emedicine.medscape.com]

• Headache

  SUNCT) and cluster headaches. [americanmigrainefoundation.org]

  […] professional commitment to helping people affected by headache. [eurekalert.org]

  Trigeminal neuralgia (TN) has been described in association with various primary headache disorders. So far, no case of TN has been reported in association with hemicrania continua (HC). [ncbi.nlm.nih.gov]

  Part three: Cranial neuralgias, central and primary facial pain and other headaches (2) Luke Bennetto, Nikunj K Patel, and Geraint Fuller.Trigeminal neuralgia and its management.BMJ 2007; 334: 201 - 205 (3) Editorial. [gpnotebook.co.uk]

• Burning Sensation

  It may also feel like an electric shock, ache or burning sensation. Laughing, talking or even lightly touching the face can trigger it. Atypical trigeminal neuralgia is characterized by constant aching, burning or stabbing pain. [aurorahealthcare.org]

  sensation and difficulty in eating. [ncbi.nlm.nih.gov]

  After the main severe pain has subsided, you may experience a slight ache or burning feeling. You may also have a constant throbbing, aching or burning sensation between attacks. [nhs.uk]

  This may be a sudden, intense flash of pain (type 1 or TN1) or a chronic, burning sensation (type 2 or TN2). [rush.edu]

• Hypesthesia

  In case 1, pain relief was immediately achieved by RFT (75°C for 90 seconds), with moderate hypesthesia. Relapse of the triggered pain occurred 6 months later, and PRF was then applied. [ncbi.nlm.nih.gov]

  History of an identifiable traumatic event to the trigeminal nerve(s), with clinically evident positive (hyperalgesia, allodynia) and/or negative (hypesthesia, hypoalgesia) signs of trigeminal nerve dysfunction. 3. [jmedicalcasereports.biomedcentral.com]

It is generally diagnosed on the basis of the patients history of pain, the triggering factors for the pain and the type of pain. No laboratory or radiologic testing is indicated on routine basis to diagnose trigeminal neuralgia. Imaging studies like CT scan or MRI are indicated to rule out tumors or multiple sclerosis. MRI is done in patients younger than 60 years of age presenting with the pain to rule out tumors and it can also reveal multiple sclerosis plaque and pontine gliomas if present [15].

Magnetic resonance angiography (MRA) is used to locate vascular compression if any, but it is not very sensitive. It should be differentiated from temporomandibular disorder [16] as pain can be triggered by movements of tongue and facial muscles.

Treatment includes both pharmacological as well as surgical approaches.

Pharmacological approach includes the following:

• Anticonvulsants: First line of treatment includes carbamazepine followed by second line medicines like oxcarbazepine, sodium valproate, gabapentin, pregabalin, phenytoin and. In some cases, clonazepam and lidocaine have also been effective [17].
• Anti-spasmodics: Muscle-relaxing agents such as baclofen can be used alone or in combination with carbamazepine.
• Antidepressant: Duloxetine is used in few cases of neuropathic pain when present along with depression [18].
• Opiates: Morphine and oxycodone along with gabapentin is effective in cases of neuropathic pain. Gallium maltolate in a cream or ointment base has been found to relieve refractory postherpetic trigeminal neuralgia [19].

Surgical approach

It is recommended when the medical line of treatment does not provide the necessary pain relief [20].

Microvascular decompression is a procedure involving relocation or removal of blood vessels that are in contact with the trigeminal root. This procedure gives the longest period of pain relief [21].
Percutaneous radiofrequency thermorhizotomy can also be useful [22].

Likewise, gamma knife radiosurgery is also useful but its effectiveness reduces as with passage of time [23].
Balloon compression is a cost-effective percutaneous surgical procedure, which is helpful in treating aged patients that cannot undergo surgery due to other health issues. It is the best option for those who have ophthalmic nerve involvement or have complains of persistent pain after microvascular decompression.

Glycerol injections involve injecting a small amount of sterile glycerol, which damages the trigeminal nerve and blocks pain signals.

Radiofrequency rhizotomy is a procedure wherein the surgeon uses an electrode to heat the selected division or divisions of the nerve. If done properly this procedure can target the exact regions of the erratic pain triggers and disable them with minimal numbness.

Trigeminal neuralgia is not a life-threatening disease, but its effects last for a long time and can be disabling. Many people have periods of remission with no pain for months or years. There is unscientific evidence that it worsens as time goes by and becomes less responsive to treatment [11].

Most cases of trigeminal neuralgia are primary or idiopathic, but the compression of trigeminal roots can cause pain due to tumours or vascular anomalies. Compressing vessels can be superior cerebellar artery or venous compressions that cause the pain [2].

Aneurysms, tumors, chronic meningeal inflammation, or other lesions may irritate trigeminal nerve roots along the pons causing symptomatic trigeminal neuralgia.

Tumor-related causes of trigeminal neuralgia commonly seen in the cerebello-pontine angle are acoustic neurinoma, arachnoid cyst [3] glioblastoma, [4] epidermoid, metastases, and lymphoma.

Vascular causes could be pontine infarctions or arteriovenous malformation or an aneurysm in the vicinity.

Inflammatory causes enlist multiple sclerosis [5], sarcoidosis, and Lyme disease neuropathy.

Adjacent dental fillings made up of different metals may trigger attacks [6]. Various triggering factors such as eating, drinking, talking, brushing teeth, shaving, smiling, coming in contact with breeze etc, can lead to this pain.

In 1956, US prevalence of TN was around 107 men and 200 women per million people [7]. Recent studies suggest the prevalence of around 1.5 cases per 10,000 people. It has been reported that around 1% of the patients having multiple sclerosis develop TN [8] and it was also seen that 2% of the patients with TN have multiple sclerosis [9].

Females are more prone to it, almost two times as compared to males. In most of the cases, the disease starts after the age of 40 years.

Trigeminal nerve is a sensory nerve and hence can produce pain. In most of the cases, no structural lesions are detected but researchers agree to the presence of venous or arterial loops at the entry of trigeminal nerve into the pons that lead to compression of the nerve and thereby to pain. This compression leads to focal demyelination of the trigeminal nerve.

Neuropathic pain is the fundamental sign of injury to minute unmyelinated and scarcely myelinated primary afferent fibers that work in processing noxious stimuli. The pain mechanisms are transformed. The damaged nerve fibres cause emphatic transmission, wherein the action potentials jump from one fibre to another [10]. Absence of inhibitory inputs from big myelinated nerve fibers plays an important role. In addition, the re-entry mechanism leads to amplification of sensory inputs.

Trigeminal neuralgia cannot be prevented, but once the patient has it, there are steps to avoid the attacks of pain by taking following measures:

• Eating soft foodstuffs.
• Eating and drinking foodstuffs that are at room temperature.
• If brushing teeth triggers attacks, clean the mouth by rinsing it with warm water after meals.
• Avoid known triggers like heat, cold, touch, loud noises etc.

Trigeminal neuralgia (TN), also known as Fothergill’s diseases [1] or tic douloureux, is a neuropathic disorder manifested by episodes of intense pain in the face rising from the trigeminal nerve. It has been described as one of the most painful conditions known to mankind so much so that it can drive the patient to commit suicide.

Trigeminal neuralgia is one of the most painful neuropathic diseases known to mankind. The pain is so much so that, it depresses the patients and gives them thoughts of committing suicide. The pain hampers the persons day to day activities like talking, chewing, washing face, smiling etc.

Physically, the patient seems to be fit but mentally the patient is frustrated due to the pain and the fear of triggering the pain. The exact cause of this condition is not known but various theories of blood vessels compressing the nerve or presence of tumors or demyelinating diseases leading to the condition have been put forth.

The treatment includes various drugs that can relieve the pain and some surgeries that may be effective if the medical line of treatment fails. Apart from medicines, patient should take care of avoiding the triggering factors like cold, heat, touch etc. to prevent the attack of pain. Alternative medicines like nutritional therapy, acupuncture, vitamin therapy etc. can be given a shot, as they might be useful in some cases.