| Trigeminal neuralgia (tic douloureux) is a very painful disorder of the portion of the fifth cranial nerve known as the trigeminal nerve that supplies sensation to the face. It is characterized by recurrent electric shock-like pains in one or more branches of the trigeminal nerve, each supplying a different portion of the face. Because the sudden, sharp pain causes the individual to wince, the condition is known as tic douloureux or painful twitch.
In most cases, no specific disease of the fifth nerve or central nervous system can be found. The cause of trigeminal neuralgia is unknown. Changes that are associated with death of nerve cells (degenerative changes) or scar tissue (fibrotic changes) have been reported in the trigeminal nerve, but may not be the cause of symptoms. In some cases, the trigeminal nerve may be compressed by a tumor or blood vessels (vascular anomaly) or damaged from dental or surgical procedures, facial injury, or infections. Pain associated with trigeminal neuralgia occasionally occurs in individuals with brain stem damage resulting from multiple sclerosis, or in individuals with blood vessel abnormalities involving the root of the fifth cranial nerve.Risk: Trigeminal neuralgia can occur at any age, but is uncommon before ages 50 to 60; the female-to-male ratio is 3 to 2 (Campbell). Individuals with hypertension or multiple sclerosis have increased risk for trigeminal neuralgia. Incidence and Prevalence: Trigeminal neuralgia is the most frequent of all the painful disorders affecting nerves (neuralgias), but is still relatively rare. US incidence is 4 to 5 per 100,000 (Campbell), with a prevalence of 15 per 100,000 (Lenaerts). Trigeminal neuralgia occurs in 4% of Americans with multiple sclerosis, when it often affects both sides of the face (bilateral) (Khoromi). Internationally, trigeminal neuralgia is rare, with an estimated prevalence of 155 per 1 million individuals (Huff). |
Source: Medical Disability Advisor
| History: Individuals report a searing or burning pain on one side of the face that occurs in lightening-like jabs in the distribution of one or more branches of the nerve (paroxysm). A paroxysm of pain usually lasts for only seconds or up to 1 to 2 minutes, but may be prolonged for 15 minutes or longer. The bouts of pain may last for days, weeks, or months, then disappear for months or even years.
The individual may occasionally rub or pinch the face or make violent movements of the face and jaw. Watering of the eye on the involved side may occur. The individual may complain of extreme sensitivity of pain or touch receptors in the skin of the face (hyperesthesia). In many cases, there is a trigger zone stimulated by movement such as chewing, talking, or yawning that sets off a typical paroxysm in the face. Lightly touching the face as in shaving or applying makeup, or even a slight breeze over the affected portion may serve as a trigger. The pain is typically restricted to one or more branches of the nerve and does not spread beyond the nerve. While the pain is occasionally bilateral, paroxysms on both sides at a time are rare. Physical exam: Physical findings in individuals with trigeminal neuralgia are typically normal. A thorough examination of the teeth, jaw, and sinuses is performed to exclude other causes of pain such as infections of the teeth and nasal sinus. The neurological exam includes an assessment of the pain site of origin and mode of spread of the painful spasm. The trigeminal nerve divides into three main branches. If the first branch (ophthalmic) is affected, shock-like pain is felt along the eye, forehead, and part of the nose. The second or middle nerve branch (maxillary) sends pain along the upper lip, teeth and gum, the side of the nose, the part of the cheek under the eye, and the lower eyelid. Pain from the third branch (mandibular) is felt in the lower lip, teeth, gum, jaw, and outer edge of the tongue. If the individual is examined during an episode of pain, involuntary twitching of the facial muscles along the affected nerve branch may be seen. Tests: An imaging test (MRI) may be done to exclude the possibility of a tumor or blood vessel compressing the trigeminal nerve, or to look for changes characteristic of multiple sclerosis. Cerebral angiogram or MRI angiography can delineate abnormalities of the blood vessels. |
Source: Medical Disability Advisor
| Initial treatment utilizes certain anticonvulsant drugs that suppress the pain, and that may shorten attacks and encourage remission. Some individuals, however, become resistant to the drug or are unable to tolerate a dose high enough to relieve the pain. Acute episodes can also be relieved with intravenous injection of an anticonvulsant drug.
When medications lose their effectiveness, surgical intervention is an alternative. At present, thermal destruction of the affected nerve branch is the preferred treatment when medication is ineffective. One operative procedure involves injecting the nerve with alcohol or glycerol. This provides temporary relief from symptoms, but pain may return as the nerve regenerates. Other surgical procedures include removing part of the nerve (partial resection) or cutting pathways leading from the nerve (medullary tractotomy). The next step attempts to destroy the nerve root using pulsed radiofrequency current (thermocoagulation, percutaneous radiofrequency trigeminal gangliolysis, RFTG), or by removing part of the nerve (percutaneous ablation). If other treatments fail, neurosurgery can be done to attempt to relieve pressure on the nerve (decompression) or cut part of the nerve (partial rhizotomy). Separating abnormal vessels from the nerve root (microvascular decompression) has become increasingly popular, and is tolerated well even in the elderly. This procedure involves separating the trigeminal nerve from the nearby artery with a piece of Teflon, or by insertion of a balloon catheter which is then inflated for 1 to 10 minutes (percutaneous balloon microcompression). Gamma-knife treatment using multiple rays of high-energy photons destroys specific portions of the trigeminal nerve root. Another new approach includes noninvasive linear accelerator radiosurgery, in which radiation is specifically directed to the root entry zone of the trigeminal nerve, guided by simultaneous MRI and CT imaging for accuracy. |
Source: Medical Disability Advisor
| Medication frequently provides relief from symptoms. The course of trigeminal neuralgia is characterized by remissions. In most individuals, the sudden attacks of pain are present for several weeks or months and then stop spontaneously. The remission may be short in duration or the pains may be absent for months or years. Attack-free intervals may become shorter as the individual ages but permanent disappearance of symptoms is rare. Trigeminal neuralgia is not fatal but frequent paroxysms may incapacitate an individual. Just the fear of an attack may limit activity. Individuals with frequent and ongoing attacks may be significantly disabled by the condition.
The outcome of various surgical approaches is unpredictable, but the pain can be so great the individual must be informed of any operations that may provide relief. Microvascular decompression is 80% effective (Lenaerts). At present, thermal destruction of the affected nerve branch is the preferred surgical method with a high success rate. Both radiofrequency trigeminal gangliolysis and percutaneous balloon microcompression are successful in resolving symptoms in 75% of individuals (Campbell). Gamma-knife radiosurgery treatment results in immediate pain resolution in 60% of individuals, and more than 75% of individuals have a greater than 50% relief of pain for the first 18 months (Lenaerts). The method is currently utilized primarily as an investigational treatment, and is not yet widely available. Noninvasive linear accelerator radiosurgery results in complete pain relief in 68% of individuals, but this is similarly not widely available (Frighetto). |
Source: Medical Disability Advisor
| Individuals with trigeminal neuralgia may benefit from physical or occupational therapy intervention for nerve desensitization therapy. Those areas of the face experiencing painful responses to pressure or temperature are rubbed with a variety of stimuli such as ice cubes, soft cotton, burlap, and terry cloth. This causes the sensory nerves to accommodate to different stimuli, thereby eliciting more normalized responses to pressure or temperature. The therapist instructs the individual to perform this process independently in conjunction with medical management. |
Source: Medical Disability Advisor
| Toxic side effects of the drugs used to control the pain can damage the liver and bone marrow. Surgical procedures can cause numbness of the face or eye that may in itself be unpleasant (anesthesia dolorosa), and may also lead to complications such as corneal abrasion.
There may be residual facial numbness, jaw weakness, or corneal numbness following radiofrequency trigeminal gangliolysis. Hearing disturbances occur in 11% of individuals following percutaneous balloon microcompression (Khoromi). |
Source: Medical Disability Advisor
| Some accommodations may be needed if the individual's job involves an activity that triggers the attacks such as headphones or protective gear that touches the face, or working outdoors or in a draft where air currents can trigger facial pain. Accommodation may be needed if the individual experiences side effects from medication. |
Source: Medical Disability Advisor
| If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case. Regarding diagnosis:
- Does individual suffer from painful paroxysms of the face, lasting from seconds to 15 minutes or longer?
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Does pain persist over days, weeks, or months, and then disappear?
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Does individual make exaggerated movements of the face and complain of sensitivity to touch and pain? Which parts of the face are affected?
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Is pain triggered by the slightest movement, such as chewing, talking, shaving, applying make-up, or having a breeze touch the face?
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Was an MRI obtained?
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Was there evidence of tumor or nerve compression?
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Is there a possibility of multiple sclerosis?
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Was an angiogram or MRI angiography done? If so, were blood vessel abnormalities found?
Regarding treatment:
- What anticonvulsant medication was used? Is dosage high enough to relieve the pain? Is there another medication, or combination of medications, that would be more beneficial?
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Is individual compliant with medication regimen?
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Has individual become resistant to medication?
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Has individual been able to tolerate dosage?
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Is use of intravenous medication required?
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Are medications no longer effective for pain control?
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Is surgical intervention required? If so, what procedure is necessary?
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Could individual have multiple sclerosis, especially if individual is a young adult?
Regarding prognosis:
- Is individual getting pain relief with current medications? Do medications need to be re-evaluated?
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Would individual benefit from surgery? Would benefits of surgery outweigh risks?
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Would individual benefit from chronic pain management at a specialized pain clinic?
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Would individual benefit from psychological counseling because of fear and anxiety?
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Has medication caused liver damage or bone marrow damage?
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Has individual experienced post-surgical complications, such as numbness of the face or eye?
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Has a corneal abrasion occurred?
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What treatment(s) will be required for complications?
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What is expected outcome of treatment(s)?
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Source: Medical Disability Advisor
| Campbell, Gordon H., and Helmi L. Lutsep. "Trigeminal Neuralgia." eMedicine. Eds. Jorge Mendizabal, et al. 16 Dec. 2004. Medscape. 26 Oct. 2004 <http://emedicine.com/neuro/topic593.htm>.Frighetto, L., et al. "Noninvasive Linear Accelerator Radiosurgery as the Primary Treatment for Trigeminal Neuralgia." Neurology 62 4 (2004): 660-662. MD Consult. Elsevier, Inc. 26 Aug. 2004 <http://home.mdconsult.com/das/journal/view/40240846-2/N/14357501?sid=294021966&source=MI>. Huff, Stephen J. "Trigeminal Neuralgia." eMedicine. Eds. Theodore Gaeta, et al. 2 Feb. 2005. Medscape. 26 Oct. 2004 <http://emedicine.com/emerg/topic617.htm>. Khoromi, Suzan, Abraham Totah, and Sally B. Zachariah. "Trigeminal Neuralgia." eMedicine. Eds. Paul L. Penar, et al. 10 Dec. 2004. Medscape. 26 Oct. 2004 <http://emedicine.com/med/topic2899.htm>. Lenaerts, Marc E., and James R. Couch. "Trigeminal Neuralgia." eMedicine. Eds. Andrew Lawton, et al. 10 Dec. 2004. Medscape. 26 Oct. 2004 <http://emedicine.com/oph/topic512.htm>. |
Source: Medical Disability Advisor
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