Trigeminal Neuralgia

Trigeminal neuralgia is a long name for a simple case of nerve inflammation of the face. Trigeminal neuralgia is a rare medical condition, occurring in about 150 per million people every year. Some believe it is extremely rare in those under the age of 50; however trigeminal neuralgia occurs in younger individuals, including children.

What is Trigeminal Neuralgia?

Trigeminal neuralgia is also known by its more traditional name, tic douloureux. It is a chronic condition involving pain in the trigeminal nerve, which is in the face. The trigeminal nerve is considered to be the 5th of 12 cranial nerves that come from the base of the brain. It has three branches: 1) one that goes to the forehead area, called the ophthalmic branch or upper branch; 2) one that goes to the midface, called the middle branch or maxillary branch; 3) one that goes to the area beneath the mouth to the chin, called the mandibular or lower branch. If you have trigeminal neuralgia or TN, you can have more than one branch be involved at the same time.

Attacks can be very extreme, causing sudden burning pain or shock-like pain to the affected area. The pain can last for as long as a few seconds to as long as 2 minutes at a time. They can come fairly close together and can be very incapacitating, both mental and physical.

As mentioned, trigeminal neuralgia involves the 5th cranial nerve. The nerve supplies the sensory components of the nerves supplying the entire face, including the mouth, lips, from the forehead to the chin. This nerve only supplies sensation to the face. It doesn’t affect the motor or movement of the muscles of the face.

There are seven major forms of trigeminal neuralgia. Some are more common than others:

  • Typical TN
  • Atypical TN
  • Pre-TN
  • Multiple sclerosis-related TN
  • Secondary TN
  • Post traumatic TN
  • Failed TN

All these forms should be differentiated from one another and from a condition known as idiopathic facial pain, which is unrelated to the fifth cranial nerve. There are also other facial pain conditions that are not considered trigeminal neuralgia.

Typical Trigeminal Neuralgia

Typical trigeminal neuralgia is the most common form of trigeminal neuralgia. It is also called classical, idiopathic or essential TN. Nearly all cases of typical trigeminal neuralgia occur because of the blood vessels that travel through narrow holes in the skull along with the trigeminal nerve. The nerves become inflamed and swell so that they compress the fifth nerve. It usually occurs at the level where the nerves and blood vessels pass from the brainstem to outside the skull because this is the narrowest spot. It can be arteries or veins causing the compression of the nerve root. There can be true compression or it can be just an indentation of the nerve causing pain in the face. In people without trigeminal neuralgia, these blood vessels do not even come in contact with the area in which the trigeminal nerve enters the facial area.

When the vessels pulse upon the trigeminal nerve root, they don’t automatically do damage to the nerve. There are, however, repetitive pulsations on the nerve that can, over time, do permanent damage. It is the superior cerebellar artery that is the most commonly responsible blood vessel causing trigeminal neuralgia but other vessels can be the cause. If trigeminal neuralgia becomes severe and unrelenting, surgery called microvascular decompression surgery is done.

Atypical Trigeminal Neuralgia

Atypical trigeminal neuralgia has a different kind of pain sensation than typical trigeminal neuralgia. The pain is on one side of the face and is a constant, severe, boring type of pain that feels as though the face is burning. In some cases, it can be a case of typical trigeminal neuralgia that begins with constant pain before going on to intermittent pain. Atypical neuralgia is thought to be due to vascular (blood vessel) compression on the fifth cranial nerve. Other doctors believe that atypical TN is simply a very severe case of typical TN.

Like typical trigeminal neuralgia, atypical TN can be treated with medications as noted later in this guide. Facial surgery to relieve the pressure on the nerve root also takes care of the problem. Certain rhizotomy surgeries will effectively treat atypical TN but the procedures can unfortunately lead to painful numbness of the face.

Pre-Trigeminal Neuralgia

This is an experience that happens days to years prior to having a full-on attack of trigeminal neuralgia. You might experience strange sensations in your face in the distribution of a branch of the trigeminal nerve. It can feel like parasthesias (tingling) of the face, a toothache or other discomfort of the face. This then goes on to full blown trigeminal neuralgia. Medications can usually take care of the pain.

Multiple Sclerosis-Related Trigeminal Neuralgia

The pain from this type of trigeminal neuralgia is identical to typical TN and it turns out that 2-4 percent of those with trigeminal neuralgia actually have multiple sclerosis at some time. The pain tends to occur in younger people than in typical TN and progresses more quickly than with typical TN. It is more common to have the pain be bilateral when it is related to multiple sclerosis.

Multiple sclerosis-related TN comes from the same thing that affects the nerves of other nerves in multiple sclerosis. That is, the nerves lose their important myelin sheath. This can happen to the fifth cranial nerve just as much as it can affect the brain and peripheral nerves of the body. Medications can be used to treat this pain and a trigeminal rhizotomy can be done if medications fail. In rare cases, the pain can be from blood vessels compressing the nerve and, in this case, decompression surgery might be used successfully.

Secondary Trigeminal Neuralgia

This is trigeminal neuralgia caused by some kind of tumor pressing on the trigeminal nerve. It can also cause weakness of the face or weakness of the chewing muscles, along with a constant aching pain. Medications may work in the beginning but ultimately fail, especially if the tumor grows. The only real treatment is to remove the tumor surgically.

Post-Traumatic Trigeminal Neuralgia

A person can have an injury to the trigeminal nerve that goes on to cause a severe pain condition. It can result from a severe trauma to the face such as is seen in a car accident, sinus trauma or dental trauma. The only real treatment for this condition is a rhizotomy procedure which cuts the nerve in order to relieve pain. After the injury, the numbness might be associated with parasthesias or phantom pain. These feelings of pain are caused by permanent damage to the trigeminal nerve. The nerve nucleus of the fifth cranial nerve becomes hyperactive and causes the phantom pain.

The pain associated with post traumatic trigeminal neuralgia is usually an aching or burning pain that is constant in nature. The pain can be worsened by certain triggers, such as exposure to cold or wind on the face. These kinds of sensations can begin to happen days to even years post-injury. The most extreme form of post-traumatic TN is called anesthesia dolorosa, in which there is ongoing severe pain and numbness in the same area.

Unfortunately, medical treatment of post-traumatic trigeminal neuralgia is usually unsuccessful with pain medications rarely working. Some people have gotten better with stimulation of the trigeminal nerve. Even brain surface stimulation has been found to be helpful.

"Failed” Trigeminal Neuralgia

Unfortunately not every case of trigeminal neuralgia gets better with medications or with surgical help. When the medications have failed and the surgical interventions are attempted without relief and the pain continues, it is called failed trigeminal neuralgia. Sometimes after surgery fails, a retrial with medications is more successful than before. Some people, on the other hand, have tried medications and have tried microvascular interventions or rhizotomy without any success. They continue to have pain and TN symptoms through to the end of their lives. Because of the repetitive destructive things done to the trigeminal nerve, these people also have elements of post-traumatic TN. Stimulation of the brain may be a last ditch effort to control the pain.

Trigeminal Nerve Symptoms

As you can see from the above descriptions of trigeminal neuralgia types, there are slight differences between the types of the disease. Most typical cases of trigeminal neuralgia have the following list of symptoms. Remember that there are three branches of the trigeminal nerve that each control the sensation of the forehead, the midface or the mandibular area.

These are the primary symptoms of typical trigeminal neuralgia:

  • Episodes of shooting or jabbing pain that feels like a serious electric shock.
  • The sensation of occasional twinges of mild pain.
  • Attacks of pain triggered spontaneously that can be triggered by touching the face, chewing, brushing the teeth or speaking
  • Pain that can last from a few seconds repetitively or last a couple of minutes
  • Episodes that last anywhere from a few days or up to a few months, sometimes with periods of no pain.
  • Pain is in the specific area supplied by one or more branches of the trigeminal nerve.
  • Pain that is affecting just on side of your face at a time.
  • Pain which is focused on one spot and then spreads to a wider pattern.
  • Attacks of TN which become more intense and more frequent over time

When the pain comes on, it feels as though you have gotten an electric shock that goes on for several seconds. Common locations of this pain are around the lips, near the eyes, on the scalp, on the forehead or on one side of the nose. You can have a sudden shock-like pain whenever you are brushing your teeth, putting makeup on, swallowing, touching the face, or even having a breeze on your face.

This is a condition that is believed to be one of the most painful conditions a person can have. While most of the time it is unilateral, some people can have it on both sides of their face at the same time. The attacks are often unrelenting and repeated for a long period of time—from a few days to many months at a time. Attacks can disappear for several months or years only to start up again. Men get the disease more often than women and it is uncommon to get it before aged 50 years.

In the days prior to getting an attack of trigeminal neuralgia, you might get prodromal symptoms like tingling or a numb feeling in the affected area. These kinds of prodromal symptoms generally feel like an aching, constant pain rather than the lancinating pain seen in true trigeminal neuralgia.

The pain of trigeminal neuralgia fortunately does not occur when the patient is asleep. Patients have what’s called type I TN if they have the stabbing, shock-like pain more than fifty percent of the time. Type II TN involves having pain more than 50 percent of the time that is aching, constant or burning. In either case, the attacks worse over time and, while not fatal, can be really debilitating. Many who have the disease quit doing daily activities because they fear a recurrence of the pain.

Causes of Trigeminal Neuralgia

The causes of trigeminal neuralgia are varied and there are some doctors who believe we haven’t yet gotten to the bottom of all cases of trigeminal neuralgia. One common cause appears to be an inflamed or enlarged blood vessel that pinches the trigeminal nerve as it leaves the brainstem and passes through the tiny hole that sends it out to provide sensation to the face. The compression can wear away at the myelin coating (protective sheath) around the nerve. Blood vessels stretch as a person ages and can pulsate in the vicinity of the nerve.

People with multiple sclerosis can get trigeminal neuralgia because losing the myelin sheath is what multiple sclerosis is all about. Tumors can press on the parts of the trigeminal nerve and trauma to the skull can damage the holes through which the nerves pass. This can cause deterioration of the nerve so that it sends out pain signals. In some cases, no cause for the disease can be found and the person suffers without optimal treatment.

Some doctors believe that trauma to the nerve results in biochemical rather than biological changes in the way the nerve works. The nerves may appear normal but may be functioning biochemically at a level that triggers pain rather than normal sensation.

Others believe that trigeminal neuralgia is caused by a bad outcome in a dental procedure, an infection that affects the nerve, other demyelinating diseases or the presence of an arterio-venous malformation.

Regardless of the cause, there appears to be a burst of excessive nerve-related energy from a damaged nerve so that all the patient feels is pain.

Diagnosis of Trigeminal Neuralgia

As there are no definitive tests for trigeminal neuralgia, the doctor needs to ask you some questions regarding your pain and do a physical examination to see if you have the disease. The doctor will ask about the type of pain, such as whether or not it comes on suddenly and is a brief, shock-like pain. They will ask you the location of the pain to see if it comes on at a location that matches with the upper, middle and lower trigeminal nerves.

They will ask about what triggers the pain. Does it occur with light cheek stimulation, cool air on your cheek or eating? There will be a neurological exam to see which branches of the trigeminal nerve are involved. In some cases, an MRI test is performed to see if the trigeminal neuralgia is caused by multiple sclerosis or by a tumor pushing on the nerve. An MRI exam may or may not identify a vessel that is pinching the nerve but, using dye that lights up the blood vessels, there is a chance that a blood vessel will be visualized and the diagnosis made. The chances that a tumor is causing trigeminal neuralgia is about 5-10 percent.

Other diseases that can mimic trigeminal neuralgia include post-herpetic neuralgia, oral surgery, tooth abscess, cluster headaches, post traumatic injury to the trigeminal nerve in sinus surgery, stroke or other types of facial trauma. These all can cause neuropathic pain, which tends to be more of a burning pain, a boring pain or a dull pain. Trigeminal neuralgia mimics quite a few other facial pain disorders so it becomes difficult to really decide whether or not the condition is related to the trigeminal nerve.

Other conditions you need to think about when you have facial pain that might or might not be caused by trigeminal neuralgia include:

  • A cracked tooth
  • Migraine headache
  • Joint pain in the lower jaw
  • Temporal arteritis, an inflammation of blood vessels in the temple
  • Idiopathic facial pain
  • Post-traumatic neuralgia—trauma to the trigeminal nerve
Specialist Survey on Trigeminal Neuralgia

A specialist in facial pain may give you the following survey to see if you have trigeminal neuralgia. In the absence of good diagnostic testing, these kinds of surveys offer the best way to know if the pain is due to trigeminal neuralgia. The more “yes” answers provided by you, the more likely it is that you have the disease:

  1. Do you have pain in your face?
  2. Do you recall the exact place you were at when this pain started?
  3. Is the pain located almost completely on a part of your face?
  4. Is the pain just on one side of the face?
  5. Do you have pain that is located deep within your ear?
  6. Is the pain located near one of your tonsils at the back of your tongue or throat?
  7. Does the pain last seconds to minutes and is it jolting, shocking or stabbing pain?
  8. Is there constant pain associated with the shocking pain, such as aching, throbbing, stinging or burning?
  9. Does the constant pain take up more than half of your waking hours?
  10. Is there constant facial numbness?
  11. Does your pain begin by having something touch your face, by washing your face, eating, shaving or brushing your teeth?
  12. Since the pain started, have you had weeks or months without the pain?
  13. Have you ever taken baclofen or an anti-seizure drug for the pain?
  14. Did any of the above medications help reduce the pain?
  15. Have you had surgery on your trigeminal nerve for the pain?
  16. Have any of the above treatments helped the pain?
  17. Did the pain start only after you had the above surgery?
  18. Did your pain start after you had a case of the shingles?
  19. Do you have multiple sclerosis?
  20. Did your pain begin after an injury?
  21. Did the pain begin after having facial surgery?
  22. When you put a finger in front of each ear and open and close your mouth, does it hurt where your fingers are?

Sometimes it just takes a preponderance of “yes” answers to diagnose trigeminal neuralgia. Other times, something like an MRI scan of the facial and brain area is necessary to determine the origin of the pain.

Treatment of Trigeminal Neuralgia

It is vitally important to take care of trigeminal neuralgia by medical or surgical means because living with this kind of pain is nearly impossible, especially if it lasts many years, even if there are breaks during that period of time.

Most doctors naturally start with medications to control trigeminal neuralgia. These include the following:

  • Topamax. This is a type of anticonvulsant that has an off label use for trigeminal neuralgia.
  • Dilantin. This is a group I antiarrhythmic and anticonvulsant that has an off label use for trigeminal neuralgia.
  • Tegretol. This is also called carbamazepine and acts also as an anticonvulsant, along with treating trigeminal neuralgia.
  • Carbatrol. This is another brand name for carbamazepine. It is indicated for seizures and for the control of pain in trigeminal neuralgia.
  • Lioresal. This is also called baclofen and is a type of muscle relaxant that has an off label use for trigeminal neuralgia.
  • Epitol. This is another brand name for carbamazepine. It works for seizure control and is indicated for the management of trigeminal neuralgia.
  • Phenytoin Sodium. This is an anti-seizure medication that also has an off label use for trigeminal neuralgia.
  • Topamax sprinkles. This is normally an anticonvulsant medication but it has an off label use for trigeminal neuralgia.

The anticonvulsant medications are generally used to block nerve firing and block the overactivity of the trigeminal nerve in cases of TN. Medications like Neurontin (gabapentin) and Baclofen are second-line agents for trigeminal neuralgia and can be used along with the anticonvulsant medications.

Antidepressant medications like tricyclic antidepressants will help you manage pain that is constant, aching, or burning. Some commonly used antidepressants include amitriptyline and nortriptyline. Interestingly, the typical anti-pain medicines like opioids tend not to help this type of pain and shouldn’t be prescribed because of their addiction potential.

Surgical Options

There are a lot of surgical procedures that can benefit those with trigeminal neuralgia. Which options are chosen depends on what the patient wants, the patient’s physical well-being, whether or not there is multiple sclerosis, where the problem in the trigeminal nerve is, and what previous surgeries can be tried. Some surgical procedures are easy and can be done as an outpatient. Others are more complex and are done using general anesthesia. The patient can expect some numbness of the face after a procedure and the trigeminal neuralgia can return to some degree after the procedure was initially successful. The main risks of surgery to correct trigeminal neuralgia include loss of hearing, stroke and postoperative infection. Balance problems can also be the result of otherwise successful surgery.

The rhizotomy is one of the main procedures for trigeminal neuralgia. It is a surgical procedure that takes certain nerve fibers and destroys them. There are several different types of rhizotomy used to treat TN:

  • Balloon compression. This procedure damages the insulation of the nerves that process light touch on the face. It is performed under general anesthesia. A tube is inserted through the patient’s cheek and is guided toward one of the branches of the trigeminal nerve. A soft catheter that has a balloon on the tip of it is inserted through the tube. The balloon is inflated to press itself against the nerve that is, in turn, pressed against the skull opening. It is kept in place for one minute and then removed. It damages the nerve and deadens it. It can be done as an inpatient or an outpatient.
  • Radiofrequency thermal lesioning. This is an outpatient procedure in which the patient is put under so that a hollow needle is passed through the cheek to where the trigeminal nerve exits the skull. The patient is then made awake and receives an electrical current to the affected area that feels just like tingling. When the tingling is felt, the patient is put under again and the nerve is heated with an electrode so that the nerves are damaged. The entire contraption is removed and the patient is finally awakened for the second time.
  • Glycerol Injection. This is done on an outpatient procedure. The patient is sedated and a small needle is passed into the cheek area next to the mouth. It is guided to where the three parts of the trigeminal nerve exit the skull. The doctor injects glycerol into the main body of the nerve. It bathes the nerve and damages the insulation of the nerve so that it is not hyperactive anymore.
  • Stereotactic radiosurgery. This procedure uses a computer as an imaging tool to send focused beams of radiation to the spot in which the trigeminal nerve leaves the brainstem. This causes a lesion to grow on the trigeminal nerve that blocks the signal’s transmission to the brain. Pain relief might take several months to actually occur. It is done as a same day procedure or the patient may stay an extra day in the hospital.
Microvascular Decompression

This is considered the most invasive of all procedures for trigeminal neuralgia. It also has the greatest chance of success when compared to the other procedures above. It is an inpatient procedure that needs to be done under general anesthesia. A small surgical opening is made behind the ear. The doctor uses a special microscope to see the nerve itself and uses a tool to push away any of the blood vessels that are pushing on the nerve. There is no postoperative numbness as in other procedures.

Patients who have this procedure need to stay in the hospital for several days after the procedure. If no vessel is found at the time of the procedure, the surgeon will cut several fibers of the nerve to block their transmission. This is called a neurectomy. The neurectomy will result in complete numbness in the area the nerves supplied. On the other hand, the nerve can grow back, resulting in a return of the agonizing pain.

Some patients choose to have complementary therapy, which can include medications plus biofeedback, acupuncture, nutritional therapy, vitamin therapy and nerve stimulation. Some of these are successful while others are less so.

Complications of Trigeminal Neuralgia

There are two types of complications to think of with trigeminal neuralgia. There are the complications of the disease itself and the complications of treating the disease.

Trigeminal neuralgia is a severe, distressing condition that often leads to extreme anxiety and depression. You might feel as though you don’t want to eat or brush your teeth for fear of triggering the pain. This, in turn, leads to poor mouth hygiene. Unless something is going on to destroy the nerve, like a tumor, there are few physical complications. Severe trigeminal neuralgia can lead to marked depression and even suicide if the patient doesn’t have any treatments that seem to help.

Trigeminal neuralgia can occur because of multiple sclerosis. In this case, other symptoms besides facial nerve pain can begin to show up such as motor problems, paralysis and numbness in other parts of the body.

Surgery for trigeminal neuralgia is not without risks. There can be anesthesia reactions, blood clots, infection and bleeding. Because there is an operation regarding the brain, there can be seizures, stroke, and occlusion to the venous sinus. The brain can swell and cerebrospinal fluid can leak from within the brain.

Most complications happen with the microvascular decompression procedure. The degree of complications depends on which nerves are cut or worked on. The major symptoms include hearing loss, loss of taste, facial paralysis, facial numbness, a hoarse voice, problems with swallowing and unsteadiness. In microvascular compression, there is a risk of death of 0.5 percent and can result in stroke. Deafness occurs at a rate of 2 percent per side of the head.

Conclusion

Trigeminal neuralgia is a rare disease that causes sharp and lancinating pain in an area of the face that lasts for several seconds and up to several minutes. It is considered one of the more painful disorders in medicine.

The trigeminal nerve is broken into three segments: the upper segment that sends pain signals from the forehead, the middle section that sends pain signals from between the yes to the top of the mouth and the lower segment, that sends pain signals from the mandibular or chin area. Usually just one segment is involved at a time; however the condition can be bilateral.

Trigeminal neuralgia has several causes. It can be due to a tumor pushing on the nerve or to blood vessels putting pressure on the nerve. In some cases, trigeminal neuralgia can be caused by a loss of the myelin sheath which causes the nerve to be damaged. This can go on to result in multiple sclerosis in some cases.

People describe the pain as sharp, stabbing or electrical in nature. It can be triggered by lightly touching the face, chewing, brushing one’s teeth or even having wind blow on the cheeks. This can result in weight loss and poor dentition because it hurts too much to brush the teeth.

There are medical treatments for trigeminal neuralgia. Medications tend to be anticonvulsant therapies or medications that cause muscle relaxation. Some people get better from the medications alone and others need to go to surgery.

There are several surgical techniques to use. Many of them block the nerve and result in numbness of the pain. The pain might come back over time. A definite surgery goes in and pushes the blood vessel away from the nerve. This is a more invasive surgery with a 0.5 percent mortality rate.

The biggest complication with trigeminal neuralgia is depression and anxiety, which can lead to suicidal behavior.

If you or a loved one has suffered trigeminal neuralgia as a result of someone else's negligence and would like to discuss your legal options with an experienced Sacramento Personal Injury Attorney, contact us online or call us at 916-921-6400 or toll-free at 800-404-5400 to set up a FREE consultation.

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