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

  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]

  In other cases, the pain is stabbing and intense but may feel like burning or prickling, rather than a shock. Sometimes the pain is a combination of shock-like sensations, migraine-like pain, and burning or prickling pain. [powerofpain.org]

  While there may be only one attack of pain, the person may experience recurrent sharp pain every few hours or every few seconds. Between the attacks, the pain resolves completely and the the person has no symptoms. [medicinenet.com]

  There is no forced contraction of the face with the pain. Patients are able to keep their face motionless during the pain if they want. [drhoney.org]

• Surgical Procedure

  BACKGROUND AND OBJECTIVE: Patients with trigeminal neuralgia (TN) and multiple sclerosis (MS) are often treated with medications or a surgical procedure. [ncbi.nlm.nih.gov]

  Surgical Treatment A surgical procedure is recommended for patients who continue to experience severe pain or side effects from medications. [irsa.org]

  Microvascular Decompression for Trigeminal Neuralgia Microvascular decompression (MVD), also known as the Jannetta procedure, is the most common surgical procedure for the treatment of trigeminal neuralgia. [neurosurgery.ucsf.edu]

  If this medication is ineffective, unsuitable or causes too many side effects, you may be referred to a specialist to discuss alternative medications or surgical procedures that may help. [nhs.uk]

• Severe Pain

  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]

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

  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]

  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]

• Epilepsy

  Vagal nerve stimulation (VNS) for refractory epilepsy is well established. [ncbi.nlm.nih.gov]

  Your GP will first prescribe a type of medicine called an anticonvulsant (usually used to treat seizures in epilepsy), which can help relieve or numb the pain in your face. [hse.ie]

  Epilepsy Radiosurgery Study Group. Predictors of efficacy after stereotactic radiosurgery for medial temporal lobe epilepsy. Neurology. 2010;74:165-172. Suh JH. Stereotactic radiosurgery for the management of brain metastases. [web.archive.org]

  Medical Treatments for Trigeminal Neuralgia The medicines doctors typically prescribe to treat trigeminal neuralgia were originally developed to treat epilepsy. [ucsfhealth.org]

  Conclusions: Catamenial Epilepsy and Catamenial Migraines have been well described in the literature but not catamenial trigeminal neuralgia. [n.neurology.org]

• Dentist

  Trigeminal neuralgia is often diagnosed by a dentist, but if you've already seen your dentist and they haven't been able to find an obvious cause of your pain, you should visit your GP. [nhsdirect.wales.nhs.uk]

  The dentist must ensure a correct diagnosis does not mistake TN as a temporomandibular disorder. [powerofpain.org]

  During that time, I saw several dentists and doctors. I had toothache-like pain, face pain and head pain. [nationalpainreport.com]

  It can feel like it is coming from one or more teeth so many people visit their dentist to begin with. [mstrust.org.uk]

  How to Get a Diagnosis The diagnosis can be made by your GP or dentist. It is crucial to give a thorough description of the pain. [tna.org.uk]

• Toothache

  This lack of pain during sleep is commonly used as a diagnostic tool when determining if a patient has TN or some other condition, like migraine or toothache. [beaumont.org]

  As it spreads, it banishes all awareness of physical discomfort — youth’s toothache as well as the neuralgia of old age. [aamboli.com]

  The pain is also commonly felt in the mouth mimicking toothache, often misleading clinicians to recommend irreversible dental treatments such as root canal therapy and extractions that do not address the source of the problem. [hoag.org]

  This nerve conveys painful sensations, for example a toothache from its origin in the dental pulp chamber into the brain, On rare occasions a malfunctioning of the nerve occurs similar to a ‘shortcircuiting’ of an electrical device. 1 What is Trigeminal [jawpain.ie]

• Jaw Pain

  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]

  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]

• 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]

• Lacrimation

  Mild autonomic symptoms such as lacrimation and/or redness of the ipsilateral eye may be present. Following a painful paroxysm there is usually a refractory period during which pain cannot be triggered. [ichd-3.org]

  […] supplied by the branches of the trigeminal ganglion are as follows (see the image above): Ophthalmic branch (V1) – This provides the sensory supply to the eyes and forehead; it exits at the superior orbital fissure, and its branches are the nasociliary, lacrimal [web.archive.org]

  The trigeminal nerve, through its branches, carries parasympathetic supply to ganglia and the lacrimal glands (V1, V2), nasal glands (V2), submandibular, sublingual, and parotid glands (V3). [academic.oup.com]

• 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]

• Fear

  Even during periods of remission, TN sufferers may live in fear of the next flare-up which inevitably occurs without treatment. The most common cause of TN is compression upon the trigeminal nerve root by one or more blood vessels. [umanitoba.ca]

  Due to the intensity of the pain during an active period, even the fear of an impending attack may prevent activity. Patients are often afraid to shave, brush their teeth or apply make-up with fear that they may precipitate an attack. [jawpain.ie]

  OBJECTIVE: Patients with trigeminal neuralgia (TN) often develop a terrible fear of triggering pain, which may lead to depression and anxiety, exerting a negative effect on their quality of life. [ncbi.nlm.nih.gov]

  Even when pain-free, you may live in fear of the pain returning. [nhs.uk]

  However, because of fear that the intense pain might return, people can be quite distraught. Trigeminal neuralgia tends not to occur when the person is asleep, and this differentiates it from migraines, which often waken the person. [medicinenet.com]

• 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]

• Facial Pain

  Your facial pain reoccurs after a percutaneous or radiosurgery procedure. Your facial pain is in the ophthalmic (1 of the trigeminal nerves) nerve or all three trigeminal nerves. [neurosurgeonsofnewjersey.com]

  Helpful Resources on Trigeminal Neuralgia and Facial Pain Read our Trigeminal Neuralgia Care Center brochure Visit the Facial Pain Association (formerly the Trigeminal Neuralgia Association) Visit Dr. Kamal’s website Read a paper by Dr. [mercycares.com]

  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]

  Introduction Trigeminal Neuralgia (TN) is a facial pain syndrome. It is typically characterised by short term, unilateral facial pain following the sensory distribution of cranial nerve V, the Trigeminal Nerve. [physio-pedia.com]

  Trigeminal neuralgia (or tic douloureux) is the most common craniofacial pain syndrome. This facial pain syndrome usually develops in individuals over 50 years old. [columbianeurosurgery.org]

• Facial Numbness

  There is also a small risk of some hearing loss, facial weakness, facial numbness, and double vision. The procedure carries a very small risk of stroke and fatality. [medicalnewstoday.com]

  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

  Introduction Trigeminal Neuralgia (TN) is a facial pain syndrome. It is typically characterised by short term, unilateral facial pain following the sensory distribution of cranial nerve V, the Trigeminal Nerve. [physio-pedia.com]

  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]

• Neuralgia

  Treatment Options for Trigeminal Neuralgia Type 1 Medication In almost every instance of trigeminal neuralgia type 1 (TGN1), medication is tried to avoid surgery. [neurosurgeonsofnewjersey.com]

  What is trigeminal neuralgia (TN)? Trigeminal neuralgia is inflammation of the trigeminal nerve, causing intense facial pain. [medicinenet.com]

  Trigeminal neuralgia must also be differentiated from migraine headaches and from other cranial neuralgias (i.e., neuralgias affecting cranial nerves other than the trigeminal). [encyclopedia.com]

  Trigeminal Neuralgia Association of Canada TNA The Facial Pain Association Lecture on Trigeminal Neuralgia I had the opportunity to give a lecture to the Trigeminal Neuralgia Association of Canada (TNAC) on Saturday March 30, 2013 via a Webinar. [drhoney.org]

• Trigeminal Neuralgia

  What is Trigeminal Neuralgia Type 1? Trigeminal Neuralgia – also called tic douloureux – is a facial pain condition that affects the largest nerve in the head, the fifth cranial (trigeminal) nerve. [neurosurgeonsofnewjersey.com]

  What is trigeminal neuralgia (TN)? Trigeminal neuralgia is inflammation of the trigeminal nerve, causing intense facial pain. [medicinenet.com]

  Trigeminal neuralgia must also be differentiated from migraine headaches and from other cranial neuralgias (i.e., neuralgias affecting cranial nerves other than the trigeminal). [encyclopedia.com]

  Trigeminal Neuralgia Association of Canada TNA The Facial Pain Association Lecture on Trigeminal Neuralgia I had the opportunity to give a lecture to the Trigeminal Neuralgia Association of Canada (TNAC) on Saturday March 30, 2013 via a Webinar. [drhoney.org]

• Headache

  SUNCT) and cluster headaches. [americanmigrainefoundation.org]

  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]

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

  References Medically reviewed by Joseph Carcione, DO; American board of Psychiatry and Neurology REFERENCE: The International Classification of Headache Disorders: 2nd edition. Cephalalgia. 2004; 24 Suppl 1:9-160. [medicinenet.com]

• Dizziness

  Side effects of these medications include nausea, drowsiness, dizziness, and mental confusion. [healthcommunities.com]

  Tegretol can cause many side effects including sleepiness, forgetfulness, confusion, drowsiness, dizziness and nausea. [irsa.org]

  Two patients experienced short and mild dizziness after the therapy, but no severe side effects were reported. [ncbi.nlm.nih.gov]

  If medication does not relieve your symptoms, or if it causes undesirable side effects such as nausea, dizziness, confusion, or severe drowsiness, we may recommend surgery. [mountsinai.org]

• Seizure

  Trigeminal neuralgia drug therapy uses some of the same medicines used for seizures. [hopkinsmedicine.org]

  When one anti-seizure drug loses effectiveness, another anti-seizure medication may be tried. More than one anti-seizure medication may be necessary to control pain in an affected individual. [rarediseases.org]

  In one of the patients, the pain disappeared within one to two days following every epileptic seizure. [ncbi.nlm.nih.gov]

  The fluctuation of estrogen plays a key role in precipitating breakthrough seizures and breakthrough headaches. [n.neurology.org]

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.