Trigeminal neuralgia

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Trigeminal neuralgia
Classification and external resources
Detailed view of trigeminal nerve, shown in yellow.
ICD-10 G50.0
ICD-9 350.1
DiseasesDB 13363
eMedicine emerg/617 
MeSH D014277

Trigeminal neuralgia (TN) or tic doloureux (also known as proeopalgia) is a neuropathic disorder of the trigeminal nerve that causes episodes of intense pain in the eyes, lips, nose, scalp, forehead, and jaw.[1] It is estimated that 1 in 15,000 people suffer from trigeminal neuralgia, although the actual figure may be significantly higher due to frequent misdiagnosis. TN usually develops after the age of 50, more commonly in females, although there have been cases with patients being as young as three years of age [2].

The condition can bring about stabbing, mind-numbing, electric shock-like pain from just a finger's glance of the cheek.


[edit] About

The pain of trigeminal neuralgia is often falsely attributed to a pathology of dental origin. "Rarely do patients come to the surgeon without having many removed, and not infrequently all, teeth on the affected side or both sides." [3] Extractions do not help. The pain is originating in the trigeminal nerve itself - often in its roots - and not in an individual nerve of a tooth, but real tooth pain may be referred to the same areas of the face as that of trigmeninal neuralgia. Because of this difficulty, many patients may go untreated for long periods of time before a correct diagnosis is made. The trigeminal nerve is the fifth cranial nerve, a mixed cranial nerve responsible for sensory data such as tactition (pressure), thermoception (temperature), and nociception (pain) originating from the face above the jawline; it is also responsible for the motor function of the muscles of mastication, the muscles involved in chewing but not facial expression. Several theories exist to explain the possible causes of this pain syndrome. Leading research indicates that it is a blood vessel - possibly the superior cerebellar artery - compressing the microvascular of the trigeminal nerve near its connection with the pons. Such a compression can injure the nerve's protective myelin sheath and cause erratic and hyperactive functioning of the nerve. This can lead to pain attacks at the slightest stimulation of any area served by the nerve as well as hinder the nerve's ability to shut off the pain signals after the stimulation ends. This type of injury may rarely be caused by an aneurysm (an outpouching of a blood vessel); by a tumor; by an arachnoid cyst in the cerebellopontine angle[4]; or by a traumatic event such as a car accident or even a tongue piercing. [1] Two to four percent of patients with TN, usually younger, have evidence of multiple sclerosis, which may damage either the trigeminal nerve or other related parts of the brain. When there is no structural cause, the syndrome is called idiopathic. Postherpetic Neuralgia, which occurs after shingles, may cause similar symptoms if the trigeminal nerve is affected.

[edit] Symptoms

People with the condition "are begging to be killed," said Kim Burchiel, M.D., professor and chairman of neurological surgery at the Oregon Health & Science University School of Medicine who sees several new TN cases a week. "I'm telling you, it's total agony." The episodes of pain may occur paroxysmally. To describe the pain sensation, patients may describe a trigger area on the face, so sensitive that touching or even air currents can trigger an episode of pain. It affects lifestyle as it can be triggered by common activities in a patient's daily life, such as eating, talking, shaving and toothbrushing. The attacks are said to feel like stabbing electric shocks, burning, pressing, crushing or shooting pain that becomes intractable. Individual attacks affect one side of the face at a time, last several seconds, hours or longer, and repeat up to hundreds of times throughout the day. The pain also tends to occur in cycles with complete remissions lasting months or even years. 10-12% of cases are bilateral, or occurring on both sides. This normally indicates problems with both trigeminal nerves since one serves strictly the left side of the face and the other serves the right side. Pain attacks typically worsen in frequency or severity over time. A great deal of patients develop the pain in one branch, then over years the pain will travel through the other nerve branches.

Signs of this can be seen in males who may deliberately miss an area of their face when shaving, in order to avoid triggering an episode. Successive recurrences may be incapacitating, and the fear of provoking an attack may make sufferers reluctant to engage in normal activities.

There is a variant of trigeminal neuralgia called atypical trigeminal neuralgia. In some cases of atypical trigeminal neuralgia, the sufferer experiences a severe, relentless underlying pain similar to a migraine in addition to the stabbing pains. This variant is sometimes called "trigeminal neuralgia, type 2"[5], based on a recent classification of facial pain[6]. In other cases, the pain is stabbing and intense, but may feel like burning or prickling, rather than a shock. Sometimes, the pain is a combination of shock-like sensations, migraine-like pain, and burning or prickling pain. It can also feel as if a boring piercing pain is unrelenting. Some recent studies suggest that ATN may be an early development of Trigeminal Neuralgia.

[edit] Treatment

There is often no cure for trigeminal neuralgia. Many people however find relief from medication, chiropractic manipulation or one of the five surgical options. Atypical trigeminal neuralgia, which involves a more constant and burning pain, is more difficult to treat, both with medications and surgery.

A recent review recommended carbamazepine as a first line treatment.[7]

If patients do not find sufficient improvement some surgical treatments may be helpful.[7] Surgery may result in varying degrees of numbness to the patient and lead occasionally to anesthesia dolorosa, which is numbness with intense pain. However, some people do find dramatic relief with minimal side effects from the various surgeries that are now available.[8]

[edit] Common hurdles to receiving treatment

Owing to the rarity of TN, many physicians and dentists are unfamiliar with the affliction's symptoms. As a result, TN is often misdiagnosed. A TN sufferer will often seek the help of numerous clinicians before a firm diagnosis is made.

Those physicians that do have experience with TN are hesitant to treat patients under the age of 30 or patients who do not show nerve compression on their MRIs as nerve compression is not the only cause of TN, it may also be caused by nerve trauma done during a dental procedure such as a root canal. Patients under the age of 30 are particularly at risk of not receiving proper medical attention, as many physicians falsely believe that one must be in the later years of life in order for pain to strike.[citation needed]

There is some evidence that points towards the need to quickly treat and diagnose TN. It is thought that the longer a patient suffers from TN, the harder it may be to reverse the neural pathways associated with the pain. Therefore it is essential that physicians are made aware of the seriousness of TN, and the level of pain that their patient is in. TN is called "The suicide disease." In fact: for those who live with Trigeminal Neuralgia for more than 3 years about half commit suicide.[citation needed]

Dentists that suspect TN should proceed in the most conservative manner possible, and should ensure that all tooth structures are "truly" compromised before performing extractions or other procedures.

Because of the hurdles noted above, it is essential for patients who believe they are suffering from TN to seek the advice of a TN specialist or neurologist if they find their primary care physician to be dismissive of their pain.

[edit] Medications

  • Anticonvulsants such as carbamazepine, oxcarbazepine, topiramate, phenytoin, or gabapentin are generally the most effective medications. Generally speaking, opiate-based analgesics offer the best relief from TN attacks. Anticonvulsant effects may be potentiated with moderate to high levels of adjuvant therapies such as baclofen and/or clonazepam. Baclofen may also help some patients eat more normally if jaw movement tends to aggravate the symptoms.
  • The pain may be treated long-term with an opioid such as Morphine, methadone, oxycodone or Duragesic in patch form, and adding clonazepam and/or baclofen.
  • Low doses of some antidepressants are thought to be effective in treating neuropathic pain, but a tremendous amount of controversy exists on this topic, and their use is often limited to treating the depression that is associated with chronic pain, rather than the actual sensation of pain from the trigeminal nerve.
  • Botox can be injected into the nerve by a physician, and has been found helpful using the "migraine" pattern adapted to the patient's special needs.
  • Patients may also find relief by having their neurologist implant a neuro-stimulator.

Many patients cannot tolerate medications for years, and an alternative treatment is to take a drug such as gabapentin and place it in an externally applied cream base by a pharmacist who compounds drugs. Also helpful is taking a "drug holiday" when remissions occur and rotating medications if one becomes ineffective.

[edit] Surgery

Surgery may be recommended, either to relieve the pressure on the nerve or to selectively damage it in such a way as to disrupt pain signals from getting through to the brain. In trained hands, surgery has been reported to have an initial success rate approaching 90 percent. However, some patients require follow-up procedures if a recurrence of the pain begins.

Of the five surgical options, the microvascular decompression is the only one aimed at fixing the presumed cause of the pain. In this procedure, the surgeon enters the skull through a 25 mm (one-inch) hole behind the ear. The nerve is then explored for an offending blood vessel, and when one is found, the vessel and nerve are separated or "decompressed" with a small pad. When successful, MVD procedures can give permanent pain relief with little to no facial numbness.

Three other procedures use needles or catheters that enter through the face into the opening where the nerve first splits into its three divisions. Excellent success rates using a cost effective percutaneous surgical procedure known as balloon compression have been reported[9]. This technique has been helpful in treating the elderly for whom surgery may not be an option due to coexisting health conditions. Balloon compression is also the best choice for patients who have ophthalmic nerve pain or have experienced recurrent pain after microvascular decompression.

Similar success rates have been reported with glycerol injections and radiofrequency rhizotomies. Glycerol injections involve injecting an alcohol-like substance into the cavern that bathes the nerve near its junction. This liquid is corrosive to the nerve fibers and can mildly injure the nerve enough to hinder the errant pain signals. In a radiofrequency rhizotomy, the surgeon uses an electrode to heat the selected division or divisions of the nerve. Done well, this procedure can target the exact regions of the errant pain triggers and disable them with minimal numbness.

[edit] Stereotactic radiation therapy

The nerve can also be damaged to prevent pain signal transmission using Gamma Knife or a linear accelerator-based radiation therapy (e.g. Trilogy, Novalis, CyberKnife). No incisions are involved in this procedure. It uses very precisely targeted radiation to bombard the nerve root, this time targeting the selective damage at the same point where vessel compressions are often found. This option is used especially for those people who are medically unfit for a long general anaesthetic, or who are taking medications for prevention of blood clotting (e.g., warfarin, heparin, aspirin). A prospective Phase I trial performed at Marseille, France, showed that 83% of patients were pain-free at 12 months, with 58% pain-free and off all medications. Side effects were mild, with 6% experiencing mild tingling and 4% experiencing mild numbness.[10]

[edit] Social consequences of trigeminal neuralgia

Most suffers of TN do not present with any outwardly noticeable symptoms, though some will exhibit brief facial spasms during an attack. As a result, it is often difficult for friends and family members of TN suffers to accept that their loved one, who was previously healthy, is now suffering from intractable pain. That doubt can be a great hindrance to the support of the patient, as friends and family, as well as physicians, will often seek a psychological root cause rather than a physiological abnormality. This is especially true of those suffering from atypical TN, who may not have any compression of the TN and in whom the sole criterion of the diagnosis may be the complaint of severe pain (constant electric-like shocks, constant crushing or pressure sensations, or a constant severe dull ache) and in this case trigeminal neuralgia still exists but is not visible to physicians because it was caused by the nerve being damaged during a dental procedure such as root canals, extractions, gum surgeries, or it may be a condition secondary to multiple sclerosis.

Many TN sufferers are confined to their homes or are unable to work because of the frequency of their attacks. It is important for friends and family to educate themselves on the severity of TN pain, and to be understanding of limitations that TN can place upon the sufferer. However, at the same time, the TN patient must be extremely proactive in furthering his or her rehabilitative efforts. Enrolling in a chronic pain support group, or seeking one-on-one counseling, can help to teach a TN patient how to adapt to the newfound affliction.

As with any chronic pain syndrome, TN not being the exception, clinical depression has the potential to set in, especially in younger patients who often are undertreated for chronic pain. Friends and family, as well as clinicians, must be alert to the signs of a rapid change in behavior, and should take appropriate measures when necessary. It must be constantly reinforced to the sufferer of TN, that treatment options do exist.

[edit] Other

In one case of trigeminal neuralgia associated with tongue-piercing, the condition resolved after the jewelry was removed.[11]

Some patients have reported a correlation between dental work and the onset of their trigeminal nerve pain.

Recently, some researchers have investigated the link between neuropathatic pain, such as TN, and coeliac disease.[citation needed]

[edit] References

  1. ^ Bayer DB, Stenger TG (1979). "Trigeminal neuralgia: an overview". Oral Surg. Oral Med. Oral Pathol. 48 (5): 393–9. doi:10.1016/0030-4220(79)90064-1. PMID 226915. 
  2. ^ Bloom, R. "Emily Garland: A young girl's painful problem took more than a year to diagnose" (PDF). 
  3. ^ Dandy, Sir Walter (1987). The Brain. The Classics of Neurology and Neurosurgery (Special ed.). Birmingham: Gryphon editions. pp. 179. 
  4. ^ Babu R, Murali R (1991). "Arachnoid cyst of the cerebellopontine angle manifesting as contralateral trigeminal neuralgia: case report". Neurosurgery 28 (6): 886–7. doi:10.1097/00006123-199106000-00018. PMID 2067614. 
  5. ^ "Neurological Surgery - Facial Pain". Oregon Health & Science University. 
  6. ^ Burchiel KJ (2003). "A new classification for facial pain". Neurosurgery 53 (5): 1164–6; discussion 1166–7. doi:10.1227/01.NEU.0000088806.11659.D8. PMID 14580284. 
  7. ^ a b Gronseth G, Cruccu G, Alksne J, et al (October 2008). "Practice parameter: the diagnostic evaluation and treatment of trigeminal neuralgia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the European Federation of Neurological Societies". Neurology 71 (15): 1183–90. doi:10.1212/01.wnl.0000326598.83183.04. 
  8. ^ Weigel, G; Casey, K. (2004). "Striking Back: The Trigeminal Neuralgia and Face Pain Handbook". Trigeminal Neuralgia Association ISBN 0-9672393-2-X. 
  9. ^ Natarajan, M (2000). "Percutaneous trigeminal ganglion balloon compression: experience in 40 patients". Neurology (Neurological Society of India) 48 (4): 330–2. PMID 11146595. 
  10. ^ Régis J, Metellus P, Hayashi M, Roussel P, Donnet A, Bille-Turc F (2006). "Prospective controlled trial of gamma knife surgery for essential trigeminal neuralgia". J. Neurosurg. 104 (6): 913–24. doi:10.3171/jns.2006.104.6.913. PMID 16776335. 
  11. ^ Gazzeri, R; Mercuri, S. & Galarza M. (2006). "Atypical trigeminal neuralgia associated with tongue piercing". JAMA 296 (15): 1840–1. doi:10.1001/jama.296.15.1840-b. PMID 17047213. 

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