Trigeminal and other neuralgias

Omega-3 polyunsaturated fatty acids (PUFA) which are found in fish oil, have been studied in a wide variety of diseases, ranging from Alzheimer’s disease to Herpes Zoster (shingles). Omega-3 PUFA have proven anti-inflammatory and neuroprotective properties and have been used to treat rheumatoid arthritis, Crohn’s disease, ulcerative colitis, psoriasis, lupus erythematosus, multiple sclerosis, as well as migraine headaches.

A new study just published in Neurology showed a strong beneficial effect of Omega-3 PUFA in the treatment of diabetic nerve damage, or diabetic sensorimotor polyneuropathy in patients with type 1 diabetes. After one year of taking 750 mg of EPA and 560 mg of DHA (two of the main omega-3 fatty acids) there was a significant improvement in the nerve function.

Omega-3 PUFA are proven to help patients with coronary artery disease, while in many other conditions, including migraines, the evidence is not as strong. However, considering that we have a very large amount of data showing a benefit in a wide variety of conditions and that Omega-3 PUFA are very safe and inexpensive, it is reasonable to try EPA with DHA for any auto-immune or inflammatory condition, as well as depression.

Eating fatty fish, such as salmon and sardines 2-3 times a week can be sufficient for general health, but those with coronary artery disease and other conditions could benefit from a daily supplement. Also, fish often contains mercury, which can cause neurological and other problems. Omax3 and prescription fish oil, Lovaza are my preferred products because they contain no mercury and are highly concentrated, requiring only 1 or 2 pills a day.

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Trigeminal neuralgia is a rare, but an extremely painful conditions. Patients compare the quality and the severity of pain to an electric shock. The underlying cause is usually compression of the trigeminal nerve by a blood vessel inside the skull and underneath the brain. Surgery to place a teflon pad between the nerve and the blood vessel is curative, but many patients can avoid surgery by using drugs such as carbamazepine, oxcarbazepine, baclofen, and other. Botox, which is approved only for one pain condition – chronic migraines, appears to help other painful conditions, including trigeminal neuralgia (TN). A single previous double-blind placebo-controlled study by Chinese doctors confirmed our clinical observation that Botox does indeed help TN.

A new report presented at the annual meeting of the American Headache Society, also by Chinese researchers describes another positive study. This study compared a single injection of Botox with two injections separated by two weeks. It is not clear what was the logic in giving a second treatment so soon after the first one since Botox effect lasts 3 months. They followed 81 patients for 6 months and both groups had more than 80% success in the first 3 months and somewhat less of an effect in the last 3 months of the study. This was not a blinded study, but placebo response is relatively low in TN, probably because of the high pain intensity. While this study was not as scientific as the first one, it does offer some additional evidence of the efficacy of Botox for TN. Botox is certainly much safer than medications, although facial asymmetry can be an unpleasant cosmetic side effect, especially if pain involves the second branch of the TN (middle of the face).

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Several of my patients with trigeminal neuralgia (TN) responded to Botox injection (although some have not). My previous post on this topic four years ago discussed a study involving 40 patients with TN, of whom 68% responded to Botox. Recently, two new cases of TN successfully treated with Botox have been reported and in the past month I’ve treated three additional patients. Two of my patients had excellent relief and one had none.

One of the case reports was presented at the recent meeting of the American Headache Society in San Diego. This was a 65-year-old woman who suffered from very severe electric shock-like pain typical of TN. She did not respond to a variety of medications, including carbamazepine (Tegretol), but did respond to Botox injections. Botox did not eliminate her pain, but the severity of it was reduced by 50% and this significantly improved the quality of her life.

The current issue of Headache contains a report of a 60-year-old man with severe TN who also did not respond to any medications. He did obtain complete relief from Botox injections and Botox has remained effective for over 2 years.

With any new treatment we usually hope to see large double-blind controlled clinical trials and eventually an FDA approval. FDA approval usually compels insurance companies pay for the treatment. Botox injections have received approval for chronic migraines, excessive sweating, twitching of muscles around the eyes (blepharospasm), and several other conditions. Unfortunately, it is not likely that Botox will receive approval for the treatment of TN because it is a relatively rare condition, which will make it difficult to conduct a large blinded trial. Fortunately, the amount of Botox needed to treat TN is much smaller than what is used for migraines, making a little more affordable. We use 100 to 200 units of Botox for chronic migraines (the FDA-approved protocol calls for 155 units injected over 31 sites) and only 20 to 50 units for TN.

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A patient of mine just emailed me about a recent segment of the TV show, The Doctors, which featured a woman whose severe chronic migraines were cured by nasal surgery. The segment was shot a few weeks after the surgery, so it is not clear how long the relief will last in her case. The surgery involved removing a contact point, which occurs in people with a deviated septum. The septum, which consists of a cartilage in the front and bone in the back, divides the left and the right sides of the nose. If the bony septum is very deviated, which often happens from an injury, it sometimes touches the side of the nose, creating a contact point between the septum and the bony side wall of the nose.
contact point headache
Several small reports by ENT surgeons have described dramatic relief of migraine headaches with the removal of the contact point. If headaches are constant, then the constant pressure of the contact point would explain the pain. However, many of the successfully treated migraine sufferers had intermittent attacks. The theory of how a contact point could cause intermittent migraines is that if something causes swelling of the mucosa (lining) of the nasal cavity, then this swelling increases the pressure at the contact point and triggers a headache. This swelling can be caused by nasal congestion due to allergies, red wine, exercise, and possibly other typical migraine triggers.

This is a good theory, but it is only a theory and the dramatic relief seen after surgery could be all due to the placebo effect. The only way to prove that contact point headaches exist and can be relieved by surgery is by conducting a double-blind study, where half of the patients undergoes surgery and the other half does not. Giving both groups sedation and bringing them to the operating room will blind the patient while the neurologist who evaluates them will also not know who was operated on and who was not, making this a double-blind study. This design is also good only in theory because those who had surgery will have bloody nasal discharge and nasal packing, thus breaking the blind.

However, despite the fact that we will not see any double-blind studies in the near future, there is one way to predict who may respond to contact point surgery. An ENT surgeon can spray a local anesthetic, such as lidocaine, around the contact point during a migraine attack and if pain goes away, then surgery is more likely to help. I would not recommend anyone having surgery without such a test.

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Trigeminal neuralgia is an extremely painful condition which causes jolts of very intense electric-like pains in the face. Fortunately, many trigeminal neuralgia sufferers respond to medications or Botox injections.

Several surgical procedures have also been used to treat this condition. One of these procedures is destruction of the trigeminal ganglion. The most effective treatment involves opening the skull and placing a teflon pad between the trigeminal nerve and the blood vessel which compresses the nerve (this procedure is called microvascular decompression). This pressure on the trigeminal nerve by an artery is the cause of pain in the majority of patients. While surgery can be truly curative, it carries a risk of serious complications and should be done only if medications and Botox injections fail. Ideally, it should be performed by a surgeon who has performed hundreds of these operations.

Besides medications, Botox, and injections to destroy the trigeminal ganglion, stereotactic radiosurgery, or gamma knife, offers another alternative to brain surgery. This treatment appears to be very effective and a new study published in Neurology suggests that this procedure is more effective if it is done early. If gamma knife radiosurgery is done within a year of the onset of pain, patients remained pain free for an average of 68 months, while if it was done more than 3 years after the onset, the relief lasted only 10 months.

Although microvascular decompression is curative, two groups of patients may opt for radiosurgery. One group consists of patients who are poor surgical candidates because they have other medical conditions which may increase the risk of complications or death. Another group include those who are afraid to have open brain surgery and who prefer to have stereotactic radiosurgery.

As a side note I should mention that gamma knife, or stereotactic radiosurgery is the treatment of choice for most cases of acoustic neuroma, a benign tumor of the vestibular nerve, a nerve going from the inner ear to the brain. Open surgery almost always leads to facial paralysis and complete loss of hearing, while gamma knife can shrink the tumor without any damage to healthy tissues.

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Trigeminal neuralgia is a very painful and debilitating condition (Here is a review article I wrote for physicians). Fortunately, it is relatively infrequent – affecting 0.3% of the population, compared to 12% afflicted by migraines. This explains relative paucity of studies of this condition. A group of neurologists at the Danish Headache Center studied 158 consecutive patients with trigeminal neuralgia (TN) seen at their center over a period of one year. They published their findings in the journal Headache.

Average age of onset of pain was 53 years. TN was more common in women than men (60% vs 40%) and more common on the right side (56%). When only one of the three branches of the trigeminal nerve were affected, the first and the second were involved in 69% of cases and the third branch (lower third of the face) alone was involved in only 7% of sufferers.

The pain of trigeminal neuralgia is described the same way by almost all sufferers – it feels like a strong electric shock. It can be triggered by chewing, brushing teeth, speaking, air movement from wind or air conditioner, and at times it occurs without any provoking factor. In this study, half of the patients reported having a more persistent but milder pain in addition to the typical stabbing, electric-like pain. One fifth of patients reported to have some tearing on the side of pain and in 17% there was some loss of sensation over the area of pain.

Treatment of TN usually begins with epilepsy drugs,such as carbamazepine (Tegretol) or oxcarbazepine (Trileptal). Although 89% of patient in this study reported some improvement, only 56% of them were taking these medications because in others they caused unacceptable side effects. Other drugs that can be helpful for TN include baclofen (a muscle relaxant) and Botox injections. I’ve treated a handful of patients with TN with Botox
and about half of them responded. Botox is injected into the area of pain, which tends to be small and only a very small amount of Botox needs to be injected. Injections of Botox are safer than any oral medication, but depending on the area injected, they can cause cosmetic side effects – asymmetric appearance of the face. Botox is approved by the FDA for chronic migraines but not TN, which means that insurance companies are not likely to pay for it. However, only about one tenth of the amount of Botox used for migraine is needed to treat TN, the cost is much lower.

The authors plan to provide additional information about this group of patients in future publications.

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A new combination of two old drugs seems to provide relief for some trigeminal neuralgia (TN) sufferers. The first line drugs for TN are epilepsy drugs, carbamazepine (Tegretol) or oxcarbazepine (Trileptal). A presentation at the last annual scientific meeting of the American Headache Society by Saudi physicians described successful use of another epilepsy/pain drug, pregabaline (Lyrica) with an antidepressant/pain drug duloxetine (Cymbalta). Both Lyrica and Cymbalta are approved by the FDA for the treatment of some pain conditions, although not TN. The doctors compared Lyrica alone with Lyrica and Cymbalta in combination in 200 patients. The combination resulted in an 80% reduction of pain, which was observed within 10 days, while Lyrica alone produced a 60% reduction that started within 20 days. The dose of Lyrica was 150 mg twice a day (after a one week build up from 75 mg twice a day) and the dose of Cymbalta was 60 mg a day.

Since both Cymbalta and Lyrica have pain relieving properties, this appears to be a rational combination of medications to use in TN and possibly other painful conditions, including various types of headaches. However, as a general rule, we try to use a single drug whenever possible to reduce the potential for side effects. TN is such a severe and debilitating condition, that it may be justified to use a combination early, especially if the first line drugs, such as oxcarbazepine fail.

In my previous posts I have described the use of intravenous medications, Botox and other invasive treatments for TN.

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Trigeminal neuralgia (TN) is an excruciatingly painful disorder which affects about one in a thousand people. Patients describe the pain of TN as an electric shock going through the face. Eating and talking often triggers the pain, so some patients become malnourished and depressed. The pain is brief, but can be so frequent and severe that it causes severe disability, weight loss, severe anxiety, and depression. The good news is that most people can obtain relief by taking drugs, such as Tegretol (carbamazepine), Trileptal (oxcarbazepine), Dilantin (phenytoin), or Lioresal (baclofen). I have successfully treated several patients who did not respond to these medications with Botox injections.

Patients who do not respond to medications or Botox injections have several surgical options available. According to a new Dutch “Nationwide study of three invasive treatments for trigeminal neuralgia” published in journal Pain shows that every year about 1% of those suffering from TN undergo surgery. Of the three most common types of surgery, percutaneous radiofrequency thermocoagulation (PRT) is by far most popular – in a three year period in Holland, 672 patients underwent PRT, 87 underwent microvascular decompression (MVD), and 39 underwent partial sensory rhizotomy (PSR). The latter two procedures a performed by neurosurgeons (MVD requires opening of the skull), while PRT is usually done by anesthesiologists (a probe is inserted through the cheek to the nerve ganglion under X-ray guidance). MVD was most effective, but caused more complications than PRT, although fewer than with PSR. More patients having PRT had to have a repeat procedure, but it was still safer than the other two. Very often the physician under-treats during the first treatment of PRT in order to avoid complications. Overall, the best initial procedure for those suffering with TN is PRT and if repeated PRTs fail, MVD can often cure this condition.

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Advances in MRI imaging have allowed visualizing the trigeminal nerve and a group of Australian researchers reported on their findings in three conditions which cause facial pain. Trigeminal nerve supplies sensation to the face and facial pain of any kind is also transmitted to the brain through this nerve. Their report, which appeared in The Journal of Pain, suggests that imaging trigeminal nerve may help in making a more accurate diagnosis, which is turn may lead to more appropriate treatment.

Trigeminal neuralgia is an extremely painful condition which is characterized by very brief electric-like pains in the face. The pain is triggered by chewing, talking, brushing teeth, touching a specific spot on the face, and at times occurring without any provocation. This condition usually results from compression of the trigeminal nerve by a blood vessel as it exits the brain stem. Treatment usually involves epilepsy drugs, such as carbamazepine (Tegretol) or oxcarbazepine (Trileptal), Botox injections, nerve destruction (radiofrequency thermocoagulation) or if nothing else works, surgery (microvascular decompression of the trigeminal nerve).

Trigeminal neuropathy also causes pain in the face, but it is less intense, usually continuous and is often burning in character. It can result from an injury to the trigeminal nerve in the periphery rather than near the brain stem. Dental procedures and facial injuries can trigger this pain. This pain tends to respond better to antidepressants, such as amitriptyline (Elavil), nortriptyline (Pamelor), protriptyline (Vivactil), and an epilepsy drug, gabapentin (Neurontin).

Temporomandibular joint disorders can result from arthritic changes in this joint, displacement of the cartilaginous disc inside the joint, or from muscle spasm and inflammation around the joint.

Using special MRI techniques the researchers discovered that patients with trigeminal neuralgia had thinning of the nerve, while those with trigeminal neuropathy had thicker trigeminal nerves than normal controls. Patients with temporomandibular disorders had normal thickness of their trigeminal nerves. This is a very useful finding, particularly when the diagnosis is not clear since some patients may have symptoms of both neuralgia and neuropathy. We often have to try several drugs before finding one that is effective and does not cause side effects, so it would be helpful to know from the start which drugs are more likely to work.

Art credit:

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Trigeminal neuralgia is an extremely painful condition that causes electric-like pain in the face. It is often misdiagnosed as a dental problem, sinus headache or another condition. The pain is very brief, just like an electric shock, but it can occur continuously and is often triggered by brushing teeth, chewing, talking, or even by wind. This is a very treatable condition and it usually responds to anti-epilepsy drugs, Botox injections and, if those fail, surgery. Many patients have periods of sudden worsening of pain and until medications or Botox begin to help they need emergency treatment for pain. Narcotics (opioids) are usually ineffective. Dr. Merritt and Cohen of the Beth Israel Hospital in New York recently described the use of intravenous antiepileptic medications for acute exacerbations of trigeminal neuralgia in the emergency department. They described 21 patients, 15 women and 6 men whose aged ranged from 33-88 and the mean age was 69 (trigeminal neuralgia is more common in the elderly). 19 received intravenous fosphenytoin (Cerebyx, a drug related to an oral drug Dilantin) 2 received levetiracetam (Keppra) with excellent relief. Side effects included double vision, dizziness, sleepiness, and itchiness with fosphenytoin and no side effects were observed in 2 who received levetiracetam. Unfortunately, the most commonly used oral drugs for trigeminal neuralgia, carbamazepine (Tegretol) and oxcarbazepine (Trileptal) are not available in an injectable form. Another epilepsy drug, divalproex sodium (Depakote) can be given intravenously (Depakene) but it does not appear to be very effective for trigeminal neuralgia.

Art credit: JulieMauskop

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Botox injections relieve pain of trigeminal neuralgia, according to a new study just published in Cephalalgia, a leading headache journal. Trigeminal neuralgia is an extremely painful condition which manifests itself by intense electric shock-like pain on one side of the face. The pain is triggered by speaking, chewing and often without any provocation. Persistent pain can lead to malnutrition from the inability to chew and to severe depression and despondency. Epilepsy drugs, such as carbamazepine (Tegretol), oxcarbazepine (Trileptal), and other types of drugs often relieve the pain, but not always and at times the drugs can cause intolerable side effects.
Research on the mechanism of action of Botox has shown that it may be blocking sensory nerves and this led me to try Botox for a few of my patients with conditions other than chronic migraines and other headaches. Several patients with post-herpetic neuralgia (shingles) and a few with trigeminal neuralgia responded very well.
This rigorous double-blind, placebo-controlled study in Cephalalgia by Chinese researchers involved 42 patents with trigeminal neuralgia, of whom 40 completed the study. Among the patients who received Botox injections, 68% had significant improvement compared to only 15% of responders in the group tht received placebo. This study strongly suggests that Botox is an effective treatment for some patients with trigeminal neuralgia. The advantage of Botox is that it has significantly fewer side effects than oral drugs.

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