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The following information of the different types of Facial Pain are from the Facial Pain Association and used with their permission.

Trigeminal Neuralgia

Trigeminal neuralgia (TN) used to also be called ‘tic douloureux’ or ‘tic convulsif’. In French, tic means “muscle twitch” or “spasm”; douloureux means “painful”. TN is an example of neuropathic pain, arising from the trigeminal nerve (the fifth cranial nerve). New cases of trigeminal neuralgia affect 4 to 5 of every 100,000 people in the United States each year. TN affects women slightly more often than men; however, there is a much higher incidence for males over 80. Peak incidence begins as 50-60 years of age and increases with age. In 60-69 year-olds, it is 17.5/100,000; in >80 year-olds, it is 25.9/100,000.

TN is a unilateral facial pain syndrome (on one side); however bilateral cases (on both sides) have been reported in 2%-5% of cases. For those with bilateral pain, one side usually precedes the onset of pain on the second side- sometimes by years.

There are three branches of the trigeminal (facial) nerve: from top to bottom, they are ophthalmic, maxillary, and mandibular. Pain in one branch is reported by 36%-42% of patients: in 17%-19%, it occurs in the maxillary or mandibular branch, and 2% solely in the ophthalmic branch. When more than one branch is involved, it is most commonly in the maxillary and mandibular branches jointly (35%), and pain in all three branches in 14% of patients. This means that pain in the lower two branches accounts for 69% of patients. This helps to explain why pain in the jaw area is confused with dental pain many times.

What does TN pain feel like?

TN episodes may start as short, mild attacks and progress and cause longer, more-frequent bouts of searing pain. TN pain is described as sudden and intense, with patient ratings of 9 out of 10, or more, although less severe attacks can occur. TN pain is often describes as stabbing, shooting, sharp, piercing or electrical in nature. Pain can occur almost anywhere between the jaw and forehead, including inside the mouth. This pain can include facial twitching (hence, the term ‘tic’).

A large proportion of people have a constant aching pain between attacks; attacks might last for longer that two minutes. Some people have eye tearing on the same side of the face. Mild sensory changes are also reported. Many people report that their TN attacks become more intense and frequent over time, sometimes with pain-free periods in between.

What are the types of TN?

The names used to differentiate types of TN may vary from doctor to doctor. In order to provide one internationally accepted naming standard, below is the International Classification of Headache Disorders 3rd Edition. The following classifications are based on a consensus between the International Headache Society (IHS) and the International Association for the Study of Pain (IASP).

Classical trigeminal neuralgia (also called TN1/Typical TN)

Description: Classical trigeminal neuralgia without persistent background facial pain.

An artery or vein compressing the trigeminal nerve causes the intense pain of TN. This type of TN is sometimes referred to as TN1, Type 1, or Classic TN. Classical TN is characterized by sharp, stabbing, paroxysms of severe pain, typically lasting a fraction of a second to two minutes. The paroxysms are very severe in intensity, usually having a trigger zone or an action that will trigger or activate the shock-like jolt. The pain is almost always unilateral (on one side) and located in the second (midface) or third (jaw) trigeminal nerve branches. Pain rarely is seen in the first division (forehead).

The cause of Classical TN is typically nerve compression by a vessel, usually the superior cerebellar artery on the trigeminal nerve root as it leaves the brain stem or pons. Classical trigeminal neuralgia with purely paroxysmal pain is also marked by periods of complete pain-free remissions.

Classical TN with concomitant continuous pain (also called Atypical TN/ATN/TN 2)

Description: Classical trigeminal neuralgia with persistent background facial pain. People with atypical TN experience a persistent dull ache or burning sensation in one part of the face. However, episodes of sharp pain can complicate atypical TN. There is often not a specific trigger point for the pain; the pain may grow worse over time.

Secondary trigeminal neuralgia

Description: Trigeminal neuralgia caused by an underlying disease.

Idiopathic trigeminal neuralgia

Description: Trigeminal neuralgia with neither electrophysiological tests nor MRI showing significant abnormalities.

Painful trigeminal neuropathy

Description: Facial pain in the distribution(s) of one or more branches of the trigeminal nerve caused by another disorder and indicative of neural damage (including herpes zoster, postherpetic neuralgia, and post-traumatic neuropathy).

Medication for trigeminal neuralgia

The medicines doctors typically prescribe to treat trigeminal neuralgia were originally developed to treat epilepsy. However, this class of medications, called anticonvulsants, has been found to be quite effective in treating nerve pain, including trigeminal neuralgia. A positive response to these drugs might signal to your doctor that classical TN is an accurate diagnosis. Carbamazepine and oxcarbazepine are frequency-dependent sodium channel blockers that reduce pain in approximately 90% of people with TN. These drugs are not always well tolerated and need to be titrated (increasing or lowering doses) carefully.

Where there is a continuous or longer lasting dull, burning, aching background pain, the addition of a tricyclic antidepressant such as nortriptyline, in doses around 50-100 mg, at bedtime, may be helpful. Other anticonvulsants such as levetiracetam and zonisamide may be useful but have not been studied in placebo-controlled trials. Baclofen is a muscle relaxant that is very effective in trigeminal neuralgia in doses between 5 and 80 mg daily. Sedation is the most significant side effect. Phenytoin may be used as an alternative in doses of 100 – 300 mg per day.

Pain triggers

Stimulus-provoked pain is typical of TN. Triggered pain is one of the signs to your doctor to indicate a diagnosis of TN. In most people, TN pain is triggered by ‘innocuous mechanical stimuli’- that would not hurt someone without TN. Subtle stimuli can be a breeze or light touch of the face. Touch plus facial movements can also trigger pain. Movement alone can also be enough to provoke TN pain. The location of your pain may be different from the location that was stimulated. You may also experience a refractory period of several seconds or minutes after a pain attack when a new attack cannot be provoked.

Attacks of TN may be triggered by:

  • Touching the skin lightly (reported in 65%)
  • Washing
  • Shaving
  • Brushing teeth
  • Blowing the nose
  • Drinking hot or cold beverages
  • Encountering a light breeze
  • Applying makeup
  • Smiling
  • Talking (reported in 76%)
  • Chewing (reported in 74%)
  • Cold (reported in 48%)

There are also reports of pain triggered by sweet, salty, or spicy foods, which also might indicate a dental issue. Your trigger may not be listed here, but that does not mean that you do not have TN.

Diagnosing TN

TN can be very difficult to diagnose, because there are no specific diagnostic tests and symptoms are very similar to other facial pain disorders. Trigeminal neuropathic pain is almost always diagnosed by your description of your symptoms. The Burchiel Questionnaire or the McGill Pain Questionnaire may help your doctors determine how to treat you for your pain.

Your doctor will likely order an MRI scan when TN is suspected in order to rule out multiple sclerosis or a tumor and to look for an offending blood vessel that is causing the pain. High-resolution, thin-slice or three-dimensional MRIs have the ability to show fine trigeminal nerve compression.

Commercial names for high-resolution images are:

  • SSFP: Steady-State Free Precession
  • GE FIESTA: Fast Imaging Employing Steady-state Acquisition
  • Siemens FISP: Fast Imaging with Steady-state Precession
  • Philips FFE: Fast Field Echo, b-FEE: Balanced Fast Field Echo
Does TN go away on its own?

It is not likely that your pain will resolve on its own. TN pain usually occurs in cycles, sometimes with periods of remission for weeks, months or even years. Over time, attacks of pain may come more frequently and be increasingly severe

Anesthesia dolorosa (AD)

Anesthesia dolorosa (AD) is a feeling of pain in an area that is completely numb to the touch. “Anesthesia dolorosa” literally means “painful numbness”. Numbness describes a loss of sensation or feeling in a part of your body, but it is often accompanied by or combined with other changes in sensation.

AD causes pain in one or more areas of the face which are completely numb to touch. The pain is described at constant, burning, aching, squeezing, heaviness, tightness, pressure or likened to pins and needles. The primary pain is usually continuous or near-continuous. You may also experience brief bursts of pain, but these are not typically the predominant pain type. Diagnosis is generally based on the description of symptoms.

AD occurs when the trigeminal nerve is damaged so that the sense of touch is diminished or eliminated while a malfunctioning sensation of pain is left intact. AD is caused by nerve damage, either from an underlying condition, traumatic injury, or from past treatment of the trigeminal nerves. AD is referred to as a deafferentation pain syndrome, meaning that it results from complete or partial interruption of nerve impulses.

Anesthesia dolorosa and trigeminal neuralgia

AD pain is usually constant with a burning or jabbing quality, while trigeminal neuralgia (TN) is intermittent, with sharp, electric-like jabs. The distinction between the two can affect the course of treatment. Further destructive procedures for an AD patient may make the condition worse.

What causes anesthesia dolorosa?

AD can be a side effect of surgery involving any part of the trigeminal system. There are several theories about what causes AD.

  • Theory 1: The touch-carrying nerve fibers are injured by surgery, while little or no damage occurs to pain-carrying fibers.
  • Theory 2: Surgical injury may also prevent nerve fibers from overlapping as they normally should, resulting in distorted signals being sent to the brain.
  • Theory 3: AD pain is much like phantom limb pain, but is occurring to an amputated trigeminal nerve branch instead of an arm or leg. After surgery, when these pain signals suddenly stop, the brain may deal with this loss of input by remembering and replaying old pain signals.
Treatment for anesthesia dolorosa

Medications

  • Muscle relaxants (Baclofen, Zanaflex)
  • Antidepressants like amitriptyline (Elavil), nortriptyline (Pamelor), clonidine (Catapres), paroxetine (Paxil)
  • Anticonvulsants such as TN- carbamazepine (Tegretol, Carbatrol), oxcarbazepine (Trileptal), gabapentin (Neurontin), clonazepam (Klonopin), valproate (Depakote), topiramate (Topamax), phenytoin (Dilantin)
  • Topical anesthetic (EMLA)
  • Topical ointments (Zostrix, Capsaicin-P)
  • Anesthetic Injections (lidocaine), opioids
  • Oral Morphine drugs (oxycontin)

Complementary health approaches

  • Acupuncture
  • Upper cervical chiropractic
  • Nutrition therapy
  • Hot and cold compresses
  • Biofeedback
  • Electrical stimulation (TENS, SCENAR)
  • Medical treatment
  • Anesthetic injections (nerve blocks)
  • Smiling
  • Motor cortex stimulation (an implanted electrode gives constant electrical stimulation)
  • Drez procedure

As medical science better understands the brain, surgeons also are looking into the newer field of stimulating or selectively disabling parts of the brain that process pain signals.

Brain tumors

Only about 1% of people with trigeminal neuralgia have their symptoms caused by a brain tumor. Pain caused by a vascular loop, or even pain caused by MS is much more common. Nevertheless, the possibility of a brain tumor is the most important reason that any person presenting with facial pain symptoms should undergo an MRI as part of the routine workup.

In the rare instance where a brain tumor is the cause, there are three types of brain tumors that may be: the first two are the two most common types of benign brain tumor: meningioma and schwannoma. Vestibular schwannomas (also known as acoustic neuromas) can cause facial numbness or pain only if they grow large enough to affect the trigeminal nerve. The third is a rare type of benign brain cyst tumor know as an epidermoid. It would be extremely rare for other types of brain tumors, including metastatic tumors and malignant brain tumors to cause trigeminal neuropathic pain. Tumors of the face involving the trigeminal nerve may also cause pain, but it would be unusual for pain caused by these tumors to be typical of neuralgia.

Diagnosis and frequency of brain tumor causing facial pain

Undergoing an MRI during diagnostic testing should show the existence of a tumor and would provide you and your doctor information about how to proceed with treatment. Neurologists and neurosurgeons understand when and how to image the trigeminal nerve whenever the diagnosis of trigeminal neuralgia is considered.

In a series of 1,185 patients who underwent surgery for TN over a period of two decades by Dr. Peter Jannetta, less than 1%, or eight patients, had an associated acoustic neuroma (AN) tumor. In a series of 2,000 patients over one decade operated on by Dr. John Tew, only four patients had an associated AN. The patients in Dr. Tew’s series had numbness in their faces caused by the tumor, and one had burning pain, not the typical stabbing pain associated with TN. These observations may indicate what treatment is likely to be more effective.

Upon review of his treatment of his eight patients with AN, Dr. Jannetta concluded that the cause of their stabbing pain was the presence of an artery that had been pushed towards the trigeminal nerve by the expanding AN. It was not the tumor causing the neuropathic facial pain; rather, it was the compression of the nerve by the force of arterial pulsations. To cause TN, an AN has to grow large enough to come into contact with the trigeminal nerve.

Treatment for brain tumor causing facial pain

If a tumor is compressing your trigeminal nerve, the goal of the surgery is to move that rtery off the nerve and maintain that separation with a small cushion- the same goal as an MVD surgery. The goal of radiation is to prevent tumor growth, not to make the tumor shrink or disappear; therefore, TN caused by a tumor will likely not be relieved. If the target is the trigeminal nerve, radiation may be an option.

There are neurosurgeons experienced treating tumors and trigeminal neuropathic pain who can discuss with you the options for treatment and help you to decide what the best one is for you.

Burning Mouth Syndrome

Burning mouth syndrome (BMS), also known as glossodynia, is a relatively rare condition that causes a burning sensation in your mouth with no obvious cause. This feeling is often compared to taking a large gulp of hot soup or coffee; it may also feel boiling, scorching, dry, tingling, rough/sandy, or stinging. BMS affects 2% of the general population; women are seven times more likely to have BMS than men. Of the women diagnosed, post-menopausal women are more likely to be diagnosed with BMS.

Symptoms of burning mouth syndrome

The burning mouth sensation is typically felt on the tip, sides, and/or top of the tongue, but can also occur on the roof of the mouth, and inside the area of the lips. You may also experience the sensation on your gums and/or inside of your cheeks. The burning can be in one or many of these areas at the same time, and can start suddenly or increase in intensity over time. The sensation may be constant or intermittent (come and go).

BMS can be quite uncomfortable and disturbing, but it is a benign condition- it does not endanger your health. The feeling is usually chronic, lasting over a period of weeks, months, or longer. BMS might follow a pattern for you- for example, it might start mildly in the morning, increasing in intensity during the day. 

Other symptoms may include:

  • Dry mouth, feeling of thirst
  • Changes in taste- bitter or metallic taste in your mouth
  • Numbness in your mouth
  • People with BMS sometimes also report headache, fatigue, pain in other parts of the body, tinnitus (noise or ringing in the ears), and anxiety. 
Causes of burning mouth syndrome

There may or may not be an underlying cause for your BMS. In attempting to get a diagnosis and treatment to relieve BMS, many people visit their dentist, general practitioner, dermatologist, ENT and other medical specialists. Your doctor will likely attempt to rule out underlying conditions such as: 

  • Vitamin deficiency, particularly vitamin B
  • Anemia 
  • Type 2 diabetes 
  • Medication side effects 
  • Allergies 
  • Oral yeast infection (thrush) 
  • Reflux 
  • Dental issues
How is burning mouth syndrome diagnosed?

In the absence of an underlying cause, research suggests that BMS can be a neuropathic pain syndrome, caused by malfunctioning nerves and may be due to injury of the trigeminal nerve, or nerve fibers in the tongue. Psychiatric disorders such as depression and PTSD may be linked to BMS. TMD, chronic fatigue syndrome, and fibromyalgia may be contributing factors. 

Your doctor will examine you and listen to your explanation of your symptoms and history. You may have tests such as blood work, oral cultures, allergy testing or tissue biopsy to rule out underlying conditions. Your doctor may refer you to a dental expert if BMS is diagnosed. 

Treatment for burning mouth syndrome

Treatment for BMS aims to lessen your symptoms. From 50% to 66% of people with BMS will experience some degree of improvement with treatment over a few weeks or months. You may have to try a number of therapies to find the best one or combination for you. Cognitive behavior interventions such as cognitive behavioral therapy can be successful at helping you minimize your discomfort with BMS. There are also at-home or homeopathic treatments you may try- remember to check with your doctor before you do. 

Medications for burning mouth syndrome

Topical therapies, such as lidocaine gel or capsaicin cream 

Avoid triggers

You may determine if certain foods or products trigger your BMS. If so, you can minimize or eliminate your exposure to them. Some common BMS triggers are:

  • spicy foods
  • hot food
  • acidic food
  • tobacco
  • mouthwash containing alcohol
Glossopharyngeal Neuralgia

Glossopharyngeal neuralgia (GPN or GN) is a relatively rare condition, occurring in less than one person in 100,000. The glossopharyngeal nerve is the 9th cranial nerve. It is responsible for feeling in the tongue and throat and movements such as swallowing and the gag reflex, along with others. “Glosso-” means related to the tongue; “pharyngeal” means related to the pharynx, or throat. The pain of GPN is typically severe, brief, sudden, and recurrent pain in the ear, base of the tongue, tonsils or beneath the angle of the jaw. Clusters of one-sided sharp, stabbing, pain shooting from throat to ear, or vice versa is characteristic of GPN. You may also feel an aching pain in the same area which persists. The pain can be present for years and may have spontaneous remissions. The pain attacks usually last for no more than two minutes. The sudden, brief episodes of GPN can be mistaken for trigeminal neuralgia.

Commonly, attacks occur during the day and can be triggered by movements of the jaw, such as swallowing, cold liquids, chewing, talking, sneezing, and clearing the throat. Touching the area and even sudden movements of the head can trigger GPN attacks. For some people, eating particular foods- sweet, spicy, or sour can trigger GPN.

Additional rare symptoms

  • Tinnitus (ringing, noise in the ear)
  • Vomiting
  • Vertigo
  • Swelling
  • Involuntary movements
Diagnosing glossopharyngeal neuralgia

Diagnosis for GPN is made through your explanation of your symptoms and an examination by your doctor. Because GPN can appear similar to trigeminal neuralgia, it is important for your doctor to exclude TN and other causes of pain due to inflammation or growths. Your doctor will also map out your pain to understand if it is typical GPN or involves other cranial nerves. Laboratory tests may be given to rule out underlying disease. MRI, MRA, and other imaging tests of the head or neck may be used to rule out nerve compression or tumors.

What causes glossopharyngeal neuralgia?

Most cases of GPN happen spontaneously or no cause is found. In some cases, it is caused by compression of the nerve by tumors, malformations, vascular compression, multiple sclerosis (MS), infection, or trauma.

How is glossopharyngeal neuralgia diagnosed? 

Your doctor will perform an examination of your head and neck, ask you to explain your symptoms and history, and order tests, including an MRI to rule out underlying causes such as trigeminal neuralgia, tumor or underlying disease.

Treatment of glossopharyngeal neuralgia
  • Medications including anti-seizure drugs such as carbamazepine, gabapentin, and pregabalin
  • Vitamin B12
  • Glossopharyngeal nerve block, which may also confirm a GPN diagnosis if successful
  • Surgery, including microvascular decompression (MVD) and rhizotomy
  • Gamma Knife (radiation) surgery
Multiple Sclerosis (MS)

MS is a chronic disease of the central nervous system. MS can include a wide variety of symptoms and signs, including numbness, paresthesias, pain, weakness, spasticity, fatigue, vertigo, visual difficulties, gait dysfunction, bladder disturbances, and cognitive changes. There appears to be a genetic component to this disease, but the cause of MS is still unknown. Scientists believe that a combination of environmental and genetic factors contribute to the risk of developing MS. The progress, severity and specific symptoms of MS in any one person cannot yet be predicted. Most people with MS are diagnosed between the ages of 20 and 50, with at least two to three times more women than men being diagnosed with the disease.

MS and facial pain

In MS, damage to the myelin coating around the nerve fibers in the central nervous system (CNS) and to the nerve fibers themselves interferes with the transmission of nerve signals between the brain, spinal cord and the rest of the body. Disrupted nerve signals cause the symptoms of MS, which vary from one person to another and over time for any given individual, depending on where and when the damage occurs.

Facial pain occurs in 1.9-6% of patients diagnosed with multiple sclerosis. In a small percentage of patients, it may be the only presenting symptom. For patients with an established diagnosis of MS, trigeminal neuralgia is the most common associated symptom. When trigeminal neuralgia (TN) is caused by MS, it is referred to as secondary trigeminal neuralgia- there is an underlying disease causing your facial pain. Trigeminal neuralgia almost always occurs on one side of the face, although in MS patients, it occurs more frequently on both sides, in about 18 percent of cases.

Facial Pain in MS is typically:

  • Sudden, intense, sharp, superficial, stabbing
  • Lasting from a second to several minute
  • May involve more than one part of the face on the same side
  • Surgery, including microvascular decompression (MVD) and rhizotomy
  • Brought on by a trigger
  • May be bilateral (on both sides)
Treatment for facial pain due to MS

Surgical therapy is controversial. The early reports found radiofrequency rhizotomy to be useful for the initial treatment of pain; however, the recurrence rate of pain is higher and the duration of pain relief may be shorter. Radiofrequency treatment for TN-MS is still the procedure of choice for most patients. Glycerol treatment may also be an option. Some numbness needs to be produced for the best and longest-lasting results. Studies of MVD surgery for patients with MS have shown a pattern: good initial pain relief with less sustained results. The majority of the FPA Medical Advisory board do not favor MVD for these patients. At the current time, radiofrequency and glycerol procedures have the clearest indications, with more information needed over time for the remaining choices.

People with MS can experience a wide variety of symptoms and signs, including numbness, parenthesis, pain, weakness, spasticity, fatigue, vertigo, visual difficulties, gait dysfunction, bladder disturbances and cognitive changes. As pain can be present in other sites in the course of MS, there are several reports of gabapentin, and lamotrigine being useful for pain in MS. In general, patients with MS are less likely to tolerate the medications used for TN.

For more information on MS, visit the National MS Society.

Occipital Neuralgia (ON)

Occipital neuralgia (ON) is a condition in which the occipital nerves, the nerves that run through the scalp, are injured or inflamed. This causes headaches that feel like severe piercing, throbbing or shock-like pain in the upper neck, back of the head or behind the ears. It is not uncommon in a facial pain practice or in a headache clinic to hear from patients about pain in their face and head that originates, focuses, or culminates in the back of the head, the region that is called occiput. The patients’ description of the pain location may – and usually does – help in making  a correct diagnosis as most nerves in the head and neck region cover very specific anatomical distributions.

The trigeminal nerve, for example, is the main provider of sensation to the entire half of one’s face. – and, similarly, the sensation in the region behind the ear and above the hairline in the back of one’s head is supplied by a very specific group of nerves:, the occipital nerves. There are three occipital nerves on each side: the greater, the lesser, and the third occipital nerves, and all of them originate from the upper cervical spinal nerve roots, mainly from the second and third cervical levels (C2 and C3).

Occipital Nerve

As the sensory information from the occiput is carried by the occipital nerves to the central nervous system, it travels through sensory ganglia and nerve roots and then enters the spinal cord in the upper part of the neck. There it is processed in the same area that is involved with sensation from the face and the rest of the head – the so-called trigemino-cervical complex. These intricate connections explain frequent overlap of the occipital pain with various migraine and headache conditions as well as some instances of occipital pain radiating into the forehead or getting aggravated by the facial pain. 

It is important to note that among many painful conditions that involve the occipital region, the true occipital neuralgia presents a very specific pain syndrome that can be successfully treated in most patients, as long it is properly diagnosed and addressed.

Occipital neuralgia pain

Occipital neuralgia (ON) is a relatively rare condition that manifests itself with pain on one or both sides of the head. Unilateral ON (on one side) is seen in 85% of cases. The pain usually starts in the back of the head and travels higher and toward the front, eventually  reaching the very top of the head (the vertex). It is described as shooting, electric-shock like, or stabbing in nature (and this in medical terminology is referred to as paroxysmal lancinating pain). Very often there is also a dull aching pain between the shooting attacks located in the same general area. 

The duration of attacks lasting from few seconds to few minutes, the severe intensity of pain, presence of either tenderness over the course of the occipital nerves or trigger points within the occipital area, as well as pain or discomfort observed with innocuous stimulation of the scalp or hair (such as hair brushing or shampooing that would not normally cause pain) are all characteristic features of ON. 

Distribution of Occipital Nerve
How is occipital neuralgia diagnosed?

Another classical feature that helps in making proper diagnosis is the improvement or disappearance of pain in response to numbing the nerve with an injection of local anesthetic in the vicinity of the nerve in question (nerve block). Such blocks are used to both diagnose and treat ON as the pain relief from a single injection may last quite long. In order to make diagnosis of ON, the patient is asked or tested for all of the above-mentioned above features, keeping in mind that other conditions that present with pain in the occipital region (migraines, cluster headaches and hemicranias, tension headaches, cervicogenic headaches that arise from dysfunction of the joints within the spinal column and neighboring cervical muscles, etc.) have to be ruled out first.

Very frequently, in order to rule out associated anatomical pathology, it is necessary to perform appropriate imaging of the head and neck – this would usually include MRI of the brain and the cervical spine. The imaging would allow detection of Chiari malformations, cervical spondylosis, vascular, and neoplastic conditions; in most ON cases the MRI studies are read as normal or almost normal.

What causes occipital neuralgia?

Interestingly enough, the exact source of pain in ON remains unknown – it is commonly accepted as a neuropathic pain condition, meaning that the underlying process is the malfunction of the nervous system. The occipital nerves, the culprit of ON, appear to be hyperactive and irritated but the reason for this irritation is often unclear. Multiple existing theories postulate compression or entrapment of the nerve or nerves anywhere along their course in the patient’s neck and head, but there is no consensus or a universally accepted understanding of the underlying pathology.

Treatment for occipital neuralgia

As with all chronic pain syndromes, the treatment of ON is administered in systematic fashion – starting from conservative measures: medications, interventions, and, ultimately, surgery. As the natural course of ON may be self-limiting and the pain may improve over time, it may be prudent to avoid risky interventions early on in the course of the disease, but medically-refractory cases (those not yielding to treatment) are often considered for invasive treatments as the pain may become disabling and making risks of interventional or surgical treatment justified.

The common initial treatments include application of cold and warm packs, massage, and physical therapy. Rest also frequently reduces the pain. 

Medication for occipital neuralgia

Among available medications, initial preference is given to conventional anti-inflammatory drugs and muscle relaxants. The next level of treatment would include those commonly used for neuropathic pain conditions- anticonvulsants and antidepressants, including gabapentin, amitriptyline, pregabalin, carbamazepine, and nortriptyline. Although useful in relieving the pain, the opioid medications are to be avoided in ON and other neuropathic pain conditions.

Nerve blocks

The nerve blocks are considered next. Your doctor may use the block or blocks for diagnostic and for therapeutic purposes. Nerve blocks may include both short- and long-lasting local anesthetics; the medications are injected in the vicinity of each suspected nerve, and as a result of injection the territory that the nerve supplies becomes temporarily numb. Along with numbness, patients experience improvement or complete relief of their ON pain, but duration of this relief tends to be longer than duration of numbness, and sometimes the pain relief may turn out to be long-lasting or even permanent. This course of events, however, is observed in only a small fraction of ON patients, and therefore the nerve blocks have to be repeated, usually with addition of corticosteroids to the local anesthetics, adding anti-inflammatory effect to the anesthesia.

Other non-surgical treatment options

Other interventional (non-surgical) ON treatment options include injections of botulinum toxin, pulsed radiofrequency treatments, and short-term electrical nerve stimulation (so called percutaneous electrical nerve stimulation or PENS). Each of these interventional modalities is able to provide significant reduction in pain intensity in a majority of ON patients, but the longevity of improvement varies from person to person and permanent pain relief is rarely seen.

Surgery for occipital neuralgia

Surgery is reserved for the most refractory patients who fail to respond to non-surgical treatments and those with intolerable pain who experience pain recurrence after the use of less invasive approaches. Although many specific surgical procedures are available for ON patients, all of them are divided into three main groups: decompression, neuromodulation, and neuro-destruction.

Decompression surgery is based on a presumption that the pain comes from the occipital nerve(s) being compressed along their course through the muscles and fascial layers with additional aggravation from neighboring arteries that are expected to travel next to the nerves. During surgery, the nerves are released at one or several points, usually by cutting the adjacent muscle and fascia, and the additional compression points from the vessels are protected by physical separation of neural and vascular structures.

In case of unsuccessful decompression or if the pain recurs due to scar formation, there is an option to interrupt transmission of painful signals or remove the hyperactive neural structures – this is accomplished by destructive interventions which include neurectomy or neurotomy, ganglionectomy, and rhizotomy that are aimed at the nerves, spinal ganglia, and spinal nerve roots, respectively. All of these interventions are considered established treatment options for ON, but the patients are expected to discuss with their surgeons the associated risks of complications and possibility of improvement, as well as contingency plans in cases of insufficient pain relief or pain recurrence.

A very different approach in treatment of ON is based on pain suppression with electrical stimulation that is delivered by an implanted device. This technique, called occipital nerve stimulation (ONS), was developed in the 1970’s and perfected to its current shape in late 1990’s. It  is now considered a standard approach to the treatment of medically-refractory ON pain. Several years ago, practice guidelines backed by a national neurosurgical society (the Congress of Neurological Surgeons) recommended ONS for ON patients based on evidence gathered through multiple peer-reviewed publications. Despite this, however, ONS remains one of those procedures that require a complicated approval process from most insurance companies.

The surgery for ONS includes implantation of one or two electrodes in the immediate vicinity of the nerve so that the electrical pulses can reach the nerve when the device is activated. During the initial testing period (the trial), the electrodes are connected to an external device to check for the degree of improvement and presence of any side effects; these temporary (externalized) electrodes are usually removed at the end of the trial.

Later on, the implantation of the permanent device involves insertion of both the electrodes and an internal pulse generator that serves as the power source and a “brain” of the ONS system. The devices available for ONS today allow patients to turn stimulation on and off, make it stronger and weaker, adjust settings, and switch between different programs based on pattern and severity of their pain.  All of this is done with an external “remote control” that communicates with the implanted generator using telemetry. Among multiple generators and systems available for ONS today there are some devices that are rechargeable and can last, with proper recharging, up to 15 years.

No surgical treatment of ON is perfect – each modality has its own set of risks and limitations – but with proper diagnostic evaluation and clear expectations of treatment it is possible to achieve lasting pain relief, so the diagnosis of ON should not be considered a lifelong burden but rather a treatable condition that can be improved and potentially cured as long as there is a well-informed patient and a team of experienced physicians and surgeons.

By Konstantin V. Slavin, MD, FAANS

Shingles and Postherpetic Neuralgia

After you have had chickenpox, the virus lies inactive in nerve tissue near your spinal cord and brain. Years later, the virus may reactivate as shingles. Postherpetic neuralgia (PHN) is the most common complication of shingles. PHN is pain resulting from a herpes zoster outbreak (shingles) along the trigeminal nerve. Postherpetic neuralgia occurs if your nerve fibers are damaged during an outbreak of shingles.

Damaged fibers cannot send messages from your skin to your brain as they normally do. Instead, the messages become confused and exaggerated, causing chronic pain. The most common area to have PHN is along the torso, but pain in the face can also occur. The sensation may be of intense burning or stabbing, and it may feel as if it is shooting along the course of the affected nerve. 

PHN typically starts during the shingles outbreak, but lasts after the rash and blisters have healed. Chicken pox causes shingles later in life. People over the age of 60 have an increased risk of shingles. Treatment for PHN does not cure it, but aims to minimize its symptoms.  

What are the symptoms of postherpetic neuralgia?

Symptoms are usually limited to the area of skin where the shingles outbreak first occurred and may include:

  • Occasional sharp burning, shooting, jabbing pain
  • Constant burning, throbbing, or aching pain
  • Extreme sensitivity to touch
  • Extreme sensitivity to temperature change
  • Itching
  • Numbness
  • Headaches
  • Pain that lasts longer than three months 
Treatment of postherpetic neuralgia

Medication can help to alleviate the pain of PHN,  including anti-seizure medications, antiviral agents, antidepressants, and opioid pain relievers. The pain of PHN can be lessened with anticonvulsants, because they are effective at calming nerve impulses and stabilizing abnormal electrical activity in the nervous system caused by injured nerves. Gabapentin, or Neurontin, and pregabalin, also known as Lyrica, are commonly prescribed to treat this type of pain. Topical patches containing lidocaine, or other pain relievers, are also very effective. 

Complementary therapies

The therapies listed are not proven to help PHN, but you may want to investigate these options.

  • Vitamin C 
  • Homeopathic treatments 
  • Acupuncture  
  • Cupping 
  • Herbal remedies 
  • Cool packs to soothe the pain  

In most cases, the pain will gradually go away. There is a small risk the pain will return intermittently, or be with you for the rest of your life. However, the majority of patients experience no postherpetic neuralgia pain within one year. 

TMJ Pain

The temporomandibular joint (TMJ) acts like a sliding hinge, connecting your jawbone to your skull. You have one joint on each side of your jaw. Pain in the temporomandibular joint may occur in 10 percent of the population and Temporomandibular Disorders (TMD) have been reported in 46.1 percent of the US population. Inflammation within the joint accounts for TMD pain. 

Common suggested factors for TMD include bruxism (teeth grinding), trauma, bite abnormalities, and emotional stressors. Chronic joint disorders are more frequently associated with painful derangement of the TMJ.  Management of TMD is usually achieved with reducing stress on the joint through exercises and splint therapy, coupled with medications such as anti-inflammatories and muscle relaxants.  

What are Temporomandibular disorders? 

Temporomandibular disorders (TMD) are a complex and poorly understood set of conditions characterized by pain in the jaw joint and surrounding tissues and limitation in jaw movements. Injuries and other conditions that routinely affect other joints in the body, such as arthritis, also affect the Temporomandibular Joint. One or both joints may be involved and, depending on the severity, can affect a person’s ability to speak, chew, swallow, make facial expressions, and even breathe. 

The exact cause of a person’s TMD is often difficult to determine. Your pain may be due to a combination of factors, such as genetics, arthritis or jaw injury. Some people who have jaw pain also tend to clench or grind their teeth (bruxism), although many people habitually clench or grind their teeth and never develop TMD. 

In most cases, the pain and discomfort associated with TMD is temporary and can be relieved with self-managed care or nonsurgical treatments. Surgery is typically a last resort after conservative measures have failed, but some people with TMD may benefit from surgical treatments. 

Symptoms of TMD

The pain of TMD is often described as a dull, aching pain, which comes and goes in the jaw joint and nearby areas. However, some people report no pain but still have problems moving their jaws.

Symptoms may include the following: 

  • pain in the jaw muscles 
  • pain in the neck and shoulders 
  • chronic headaches 
  • jaw muscle stiffness 
  • limited movement or locking of the jaw 
  • ear pain, pressure, fullness, ringing in the ears (tinnitus) 
  • painful clicking, popping or grating in the jaw joint when opening or closing the mouth 
  • a bite that feels “off” 
  • dizziness 
  • vision problems 
Treatments for TMD

Most people with TMD have relatively mild or periodic symptoms which may improve on their own within weeks or months with simple home therapy. Self-care practices, such as eating soft foods, applying ice or moist heat, and avoiding extreme jaw movements (such as wide yawning, loud singing, and gum chewing) are helpful in easing symptoms. According to the NIH, because more studies are needed on the safety and effectiveness of most treatments for jaw joint and muscle disorders, experts strongly recommend using the most conservative, reversible treatments possible. 

Conservative treatments do not invade the tissues of the face, jaw, or joint, or involve surgery. Reversible treatments do not cause permanent changes in the structure or position of the jaw or teeth. Even when TM disorders have become persistent, most patients still do not need aggressive types of treatment.

For more information about TMD, contact the TMJ Association.  

Neuropathic Facial Pain or Dental Pain

Neuropathic pain is a chronic pain condition, and refers to all pain initiated or caused by a lesion or dysfunction in the nervous system. Trigeminal neuralgia, atypical odontalgia (phantom tooth pain), burning mouth syndrome, traumatic neuropathies, postherpetic neuralgias, and complex regional pain syndrome are neuropathic pain conditions in the orofacial region. When you first experience pain in your face, it makes sense that you or your doctor will suspect a dental issue to be the cause of the pain. In order to get a correct diagnosis quickly, it is imperative to determine if you have a dental issue or neuropathic facial pain.

Classical trigeminal neuralgia is attributable to pathological blood vessel-nerve contact in the trigeminal nerve root entry zone to the brain stem. The typical pain symptoms are characterized by sudden stabbing pain attacks.  Atypical odontalgia, also known as phantom tooth pain, or neuropathic orofacial pain, is characterized by chronic pain in a tooth or teeth, or in a site where teeth have been extracted or following endodontic treatment, without an identifiable cause. Over time, the pain may spread to involve wider areas of the face or jaws. 

The pain is called “atypical” because it is a different type of pain than that of a typical toothache. Typical toothache comes and goes and is aggravated by exposure of the tooth to hot or cold food or drink, and/or by chewing or biting on the affected tooth. There is an identifiable cause, such as decay, periodontal disease, or injury to the tooth and the pain is predictably relieved by treatment of the affected tooth.  

Atypical odontalgia 

With atypical odontalgia, the pain is described as a constant throbbing or aching in a tooth, teeth, or extraction site that is persistent and unremitting, and which is not significantly affected by exposure to hot or cold food or drink, or by chewing or biting. The pain may or may not be relieved by the injection of local anesthetic.  The intensity of the pain can vary from very mild to very severe. 

There is typically no identifiable cause to explain the pain and it often follows or is associated with a history of some type of dental procedure such as having a root canal or tooth extraction. On occasion, the pain can occur without any reason. The pain is felt in a tooth or teeth and persists in spite of treatment aimed to relieve the pain such as a filling, a root canal, or even an extraction. This often presents a frustrating and confusing situation for both the patient and the dentist, and can lead to more and more dental treatment, none of which is effective at relieving the pain. 

The diagnosis of atypical odontalgia is made after a thorough history, clinical examination, and radiographic assessment fail to identify a cause for the pain. Once the diagnosis is made, medications can be used in an effort to reduce the level of pain. 

What causes atypical odontalgia?

The cause of atypical odontalgia is not known, and therefore, some clinicians refer to the pain as “idiopathic”. In all likelihood, it is probably due to a variety of factors which may include genetic predisposition, age, and sex. It is more common in women than in men, and is found most often in the middle-aged to older age group. Some studies have found an association between atypical odontalgia and depression and anxiety, however, the significance of this association is unclear. The actual pathologic mechanism seems to be dysfunction or “short-circuiting” of the nerves that carry pain sensations from the teeth and jaws that is triggered by some type of dental or oral manipulation. Areas of the brain that process pain signals appear to undergo molecular and biochemical changes that result in a persistent sensation of pain in the absence of an identifiable cause of the pain. 

Atypical odontalgia is treated by using a variety of medications. Many different medications have been used to treat this condition; however, the tricyclic antidepressants are used most frequently. In addition to the tricyclics, other drugs used to treat chronic pain conditions, such as gabapentin, baclofen, and duloxetine may be prescribed. Generally, treatment is successful in reducing the pain but not eliminating it completely. 

Since the exact cause of this problem is not known, it is difficult to say whether this is a permanent condition. There are cases in which the pain goes away spontaneously as well as cases in which the pain gradually subsides and disappears after prolonged treatment with medications. There are many cases however, that persist and require the continued use of medications. Diagnosis and treatment are best done by a dentist with advanced training and familiarity with the problem, such as a specialist in oral medicine or orofacial pain. 

Neuropathic causes of mouth pain

Glossopharyngeal neuralgia (GPN or GN) is a relatively rare condition, occurring in less than one person in 100,000. The glossopharyngeal nerve is the 9th cranial nerve. It is responsible for feeling in the tongue and throat and movements such as swallowing and the gag reflex, along with others. “Glosso-” means related to the tongue; “pharyngeal” means related to the pharynx, or throat. The pain of GPN is typically severe, brief, sudden, and recurrent pain in the ear, base of the tongue, tonsils or beneath the angle of the jaw. Read more

Trigeminal neuralgia episodes may start as short, mild attacks and progress and cause longer, more-frequent bouts of searing pain. TN pain is described as sudden, intense, “stabbing” or “shock-like”. This pain can occur almost anywhere between the jaw and forehead, including inside the mouth. This pain is usually only on one side of the face, and can include facial twitching (hence, the term ‘tic’). A constant aching, burning feeling that may also occur before evolving into the spasm-like pain. Many people report that their TN attacks become more intense and frequent over time, sometimes with pain-free periods in between. TN is often confused with dental pain. Read more

Burning mouth syndrome (BMS), also known as glossodynia, is a relatively rare condition that causes a burning sensation in your mouth with no obvious cause. This feeling is often compared to taking a large gulp of hot soup or coffee; it may also feel boiling, scorching, dry, tingling, rough/sandy, or stinging. Read more