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Trigeminal neuralgia is a chronic pain disorder that affects the trigeminal nerve (fifth cranial nerve), which is one of the most widely distributed nerves in the head. It is mainly of two main types: typical and atypical trigeminal neuralgia. The typical form of trigeminal neuralgia results in sudden, severe, shock-like pain in one side of the face that may last from few seconds to a few minutes. The atypical form of trigeminal neuralgia results in a constant burning pain that is less severe. Episodes of pain may be triggered by any tactile stimulus to the face. Even in the same person, both forms of trigeminal neuralgia can be seen. It is one of the most painful conditions and associated with causing depression and functional disability.

At times described as the most excruciating pain known to humanity, the pain of trigeminal neuralgia typically involves jaw and lower part of face, although area around nose and above the eyes can also be involved.

The trigeminal nerve is one of twelve pairs of cranial nerves that are attached to the brain. The nerve has three branches that carry sensations from the face (upper, middle, and lower portions), as well as the oral cavity, to the brain. The ophthalmic, or upper, branch is responsible for providing sensations to most of the scalp, forehead, and front of the head. The maxillary, or middle, branch is responsible for supplying the sensations to the cheek, upper jaw, top lip, teeth and gums, and to the side of the nose. The mandibular, or lower, branch is responsible for supplying the sensations to the lower jaw, teeth and gums, and bottom lip. Trigeminal neuralgia can affect more than one nerve branch. In rare cases, both sides of the face may be affected at different times in the same individual, or even more rarely at the same time (called bilateral trigeminal neuralgia). More commonly seen in people over age 50, trigeminal neuralgia can occur at any age, including infancy. Approximately 12 per 100,000 people per year are affected; the disorder is found to be more common in women than in men.

Trigeminal neuralgia is associated with a variety of different conditions. It can be caused by a compression effect on the trigeminal nerve, by the blood vessels, as it exits the brain stem. This compression causes damage to the myelin sheath (protective coating around the nerve).

Trigeminal neuralgia may also occur in people with multiple sclerosis (demyelinating disease in which the myelin sheath of nerve cells in the brain and spinal cord are damaged). Symptoms may be caused by nerve compression from a tumor, or arteriovenous malformation. Injury to the trigeminal nerve (sinus surgery, oral surgery, stroke, facial trauma) may also produce neuropathic facial pain.

Pain of trigeminal neuralgia varies, and may range from stabbing to aching, burning sensation. The intense flashes of pain can be precipitated by vibration or contact with the cheek (shaving, washing the face, applying makeup on the face), brushing teeth, chewing, eating, drinking, talking, or being exposed to the strong wind. The pain is sudden and severe in nature, and may affect a small area of the face or may spread. Bouts of pain rarely occur when the affected individual is sleeping.

These pain attacks stop for a period of time and then return, but the medical condition can be progressive. The attacks generally worsen over time, with fewer and shorter pain-free periods in between the active episodes. Eventually, the medication to control the pain becomes less effective and the pain-free intervals disappear totally.

The diagnosis is based mainly on the person’s history and description of symptoms, along with findings of complete physical and neurological examinations. Magnetic resonance imaging (MRI) scan should be conducted to rule out a tumor or multiple sclerosis as the underlying cause. Special MRI imaging scans can reveal the presence, nature and severity of nerve compression by a blood vessel.

Treatment options of trigeminal neuralgia include medicines, surgery, and complementary methods of management. Anticonvulsant medicines that block the rapid firing of nerves are generally effective in treatment of type 1 trigeminal neuralgia. These are relatively less effective in treating trigeminal neuralgia type 2 and the examples include carbamazepine, oxcarbazepine, gabapentin, pregabalin, clonazepam, topiramate, phenytoin, lamotrigine, and valproic acid.

Tricyclic antidepressants can be used to treat pain including amitriptyline or nortriptyline. Common analgesics are not helpful but some individuals with trigeminal neuralgia type 2 do respond to opioids. Eventually, if medication fails to relieve pain then surgical treatment may be indicated. Depending on the nature and severity of pain, several neurosurgical procedures are available to treat the condition.

Rhizotomy is a surgical procedure in which nerve fibers are damaged to block pain. Balloon compression is another procedure, performed in outpatient setting that works by damaging the myeling sheath on nerves that are involved with the sensation of light touch on the face. Glycerol injection is also an outpatient procedure performed to relieve the symptoms. Radiofrequency thermal lesioning is the most often performed on an outpatient setting. Stereotactic radiosurgery (cyber knife, gamma knife) uses highly focused beams of radiation on the affected nerve that disrupts the transmission of sensory signals to the brain. Microvascular decompression is the most invasive of all trigeminal neuralgia surgeries, and offers the lowest probability of return of symptoms. In the procedure of neurectomy (partial nerve section), cutting a part of the nerve may be performed near the entrance point of the nerve at the brain stem. Complementary approaches whose therapeutic benefits have not been bolstered by medical research include exercise, yoga, creative visualization, aroma therapy, acupuncture, upper cervical chiropractic, biofeedback, botulinum injection, vitamin therapy, and nutritional therapy.