Trigeminal Neuralgia

What is trigeminal neuralgia?

Trigeminal neuralgia (TN), also called tic douloureux, is a chronic  neuropathic pain condition that affects the trigeminal or 5th cranial nerve, one of the most widely distributed nerves in the head and face. Rarely, in about 10-20% cases, both sides of the face may be affected at different times in an individual, or even more rarely at the same time (called bilateral TN) and that could be a sign of underlying disease like Multiple sclerosis, tumors of the brain. Pain attacks generally worsen in frequency or severity over  time.

What causes trigeminal neuralgia?

TN is associated with a variety of conditions. TN can be caused by a blood vessel pressing on the trigeminal nerve as it exits the brain stem. This compression causes the wearing away or damage to the protective coating around the nerve (the myelin sheath). TN symptoms can also occur in people with multiple sclerosis, a disease that causes deterioration of the trigeminal nerve’s myelin sheath. Rarely, symptoms of TN may be caused by nerve compression from a tumor, or a tangle of arteries and veins called an arteriovenous malformation. Injury to the trigeminal nerve (perhaps the result of sinus surgery, oral surgery, stroke, or facial trauma) may also produce neuropathic facial pain.

What are the symptoms of trigeminal neuralgia?

Pain varies, depending on the type of TN, and may range from sudden, severe, and stabbing to a more constant, aching, burning sensation. Neuralgia can cause causes episodes of intense, stabbing electric shocks like, burning, pressing, crushing or shooting pain in the eyes, lips, nose, scalp, forehead, and jaw. The intense flashes of pain can be triggered by vibration or contact with the cheek (such as when shaving, washing the face, or applying makeup), brushing teeth, eating, drinking, talking, or being exposed to the wind. The pain may affect a small area of the face or may spread. Bouts of pain rarely occur at night, when the affected individual is sleeping.

TN is typified by attacks that stop for a period of time and then return, but the condition can be progressive. The attacks often worsen over time, with fewer and shorter pain-free periods before they recur. Eventually, the pain-free intervals disappear and medication to control the pain becomes less effective. The disorder is not fatal, but can be debilitating. Due to the intensity of the pain, some individuals may avoid daily activities or social contacts because they fear an impending attack.

The intensity of pain can be physically and mentally incapacitating. The Affected areas on the face can be so sensitive that touching or even air currents can trigger an episode of pain. The painful attacks are said to TN can have severe affects on lifestyle as simple things such as eating, talking, shaving, and brushing your teeth can be painful. This disease before the advent of medications and other treatments was called as the Suicidal disease as the patients used to commit suicide to get rid of the obnoxious pain.

Patients often are misdiagnosed as having dental origin pain and get multiple dental procedures like extractions and Root canals done, often to relieve the pain.

Who is affected?

Trigeminal neuralgia occurs most often in people over age 50, although it can occur at any age, including infancy. The possibility of TN being caused by multiple sclerosis increases when it occurs in young adults. The incidence of new cases is approximately 12 per 100,000 people per year; the disorder is more common in women than in men.

How is TN diagnosed?

TN diagnosis is based primarily on the person’s history and description of symptoms, along with results from physical and neurological examinations.

Other disorders that cause facial pain should be ruled out before TN is diagnosed. Some disorders that cause facial pain include post-herpetic neuralgia (nerve pain following an outbreak of shingles), cluster headaches, and temporomandibular joint disorder (TMJ, which causes pain and dysfunction in the jaw joint and muscles that control jaw movement).  Because of overlapping symptoms and the large number of conditions that can cause facial pain, obtaining a correct diagnosis is difficult, but finding the cause of the pain is important as the treatments for different types of pain may differ.

Most people with TN eventually will undergo a magnetic resonance imaging (MRI) scan to rule out a tumor or multiple sclerosis as the cause of their pain. This scan may or may not clearly show a blood vessel compressing the nerve. Special MRI imaging procedures can reveal the presence and severity of compression of the nerve by a blood vessel.

A diagnosis of classic trigeminal neuralgia may be supported by an individual’s positive response to a short course of an antiseizure medication. Diagnosis of TN2 is more complex and difficult, but tends to be supported by a positive response to low doses of tricyclic antidepressant medications (such as amitriptyline and nortriptyline), similar to other neuropathic pain diagnoses.

How is trigeminal neuralgia treated?

Treatment options include medicines, surgery, and complementary approaches.

Medications:

Anticonvulsant medicines—used to block nerve firing—are generally effective in treating TN. These drugs include carbamazepine, oxcarbazepine, topiramate, gabapentin, pregabalin, clonazepam, phenytoin, lamotrigine, and valproic acid.

Tricyclic antidepressants such as amitriptyline or nortriptyline can be used to treat pain.

Common analgesics and opioids are not usually helpful in treating the sharp, recurring pain caused by TN1, although some individuals with TN2 do respond to opioids.

Eventually, if medication fails to relieve pain or produces intolerable side effects such as cognitive disturbances, memory loss, excess fatigue, bone marrow suppression, or allergy, then surgical treatment may be indicated.

Since TN is a progressive disorder that often becomes resistant to medication over time, individuals often seek surgical treatment.

Surgery

Several neurosurgical procedures are available to treat TN, depending on the nature of the pain; the individual’s preference, physical health, blood pressure, and previous surgeries; presence of multiple sclerosis, and the distribution of trigeminal nerve involvement (particularly when the upper/ophthalmic branch is involved). Some procedures are done on an outpatient basis, while others may involve a more complex operation that is performed under general anesthesia. Some degree of facial numbness is expected after many of these procedures, and TN will often return even if the procedure is initially successful. Depending on the procedure, other surgical risks include hearing loss, balance problems, leaking of the cerebrospinal fluid (the fluid that bathes the brain and spinal cord), infection, anesthesia dolorosa (a combination of surface numbness and deep burning pain), and stroke, although the latter is rare.

• Microvascular decompression (MVD) is the most invasive of all surgeries for TN, but also offers the lowest probability that pain will return. About half of individuals undergoing MVD for TN will experience recurrent pain within 12 to 15 years.  This inpatient procedure, which is performed under general anesthesia, requires that a small opening be made through the mastoid bone behind the ear. While viewing the trigeminal nerve through a microscope or endoscope, the surgeon moves away the vessel (usually an artery) that is compressing the nerve and places a soft cushion between the nerve and the vessel. Individuals generally recuperate for several days in the hospital following the procedure, and will generally need to recover for several weeks after the procedure.

A rhizotomy (rhizolysis) is a procedure in which nerve fibers are damaged to block pain. A rhizotomy for TN always causes some degree of sensory loss and facial numbness. Several forms of rhizotomy are available to treat trigeminal neuralgia:

• Balloon compression works by injuring the insulation on nerves that are involved with the sensation of light touch on the face.
• Glycerol injection is also generally an outpatient procedure in which the individual is sedated with intravenous medication.
• Radiofrequency thermal lesioning (also known as “RF Ablation” or “RF Lesion”) is most often performed on an outpatient basis.
• Stereotactic radiosurgery (Gamma Knife, Cyber Knife) uses computer imaging to direct highly focused beams of radiation at the site where the trigeminal nerve exits the brain stem.

A neurectomy (also called partial nerve section), which involves cutting part of the nerve, may be performed near the entrance point of the nerve at the brain stem during an attempted microvascular decompression if no vessel is found to be pressing on the trigeminal nerve.

Complementary approaches

Some individuals manage trigeminal neuralgia using complementary techniques, usually in combination with drug treatment. These therapies offer varying degrees of success. Some people find that low-impact exercise, yoga, creative visualization, aroma therapy, or meditation may be useful in promoting well-being. Other options include acupuncture, upper cervical chiropractic, biofeedback, vitamin therapy, and nutritional therapy. Some people report modest pain relief after injections of botulinum toxin to block activity of sensory nerves.

Chronic pain from TN is frequently very isolating and depressing for the individual. Conversely, depression and sleep disturbance may render individuals more vulnerable to pain and suffering. Some individuals benefit from supportive counseling or therapy by a psychiatrist or psychologist. However, there is no evidence that TN is psychogenic in origin or caused by depression, and persons with TN require effective medical or surgical treatment for their pain.