Trigeminal Neuralgia

What Is It, Causes, Signs, Symptoms, and More

Author:Lily Guo

Editors:Alyssa Haag, Ian Mannarino, MD, MBA,Kelsey LaFayette, DNP, ARNP, FNP-C

Illustrator:Jessica Reynolds, MS

Copyeditor:Stacy Johnson, LMSW

What is trigeminal neuralgia?

Trigeminal neuralgia, also referred to as tic douloureux, prosopalgia, or Fothergill disease, is a neuropathic disorder characterized by episodes of intense pain in the face. It is classified as a cranial nerve disorder, specifically of the trigeminal nerve (i.e., cranial nerve V). There are two trigeminal nerves (i.e., left and right), each of which is divided into three subdivisions: V1, V2, and V3, to provide innervation to one-half of the face. V1, known as the ophthalmic branch, innervates the eyes, the skin of the upper face, and the skin of the anterior scalp. V2, the maxillary branch, innervates the upper lip, teeth, gingiva, anterior soft palate, cheeks, and maxillary sinus. V3, or the mandibular branch, innervates parts of the lower jaw, tongue, lower lip, and chin. Trigeminal neuralgia commonly. Other less common locations where the pain may originate include the forehead, ear, eye, lips, nose, scalp, mouth, and side of the face. There are two types of trigeminal neuralgia: the classic type, which is the most common, occurring in up to 95% of cases, and the secondary type, also referred to as trigeminal neuralgia type II. Altogether, trigeminal neuralgia is typically a severe and debilitating condition.

Three subdivisions of the trigeminal nerve on the face.

What causes trigeminal neuralgia?

The exact cause of trigeminal neuralgia is not fully understood; however, hypothesized causes depend on the subtype. For example, it is postulated that the compression of the trigeminal nerve causes classic trigeminal neuralgia by a vascular structure (e.g., vein or artery) where it enters the brainstem. Compression of the nerve leads to  demyelination, or loss of the myelin sheath, of the nerve fibers. Damage to the myelin sheath, which is responsible for insulating nerve cells and fibers, results in increased electrical activity in the trigeminal nerve, thereby triggering pain. Conversely, secondary trigeminal neuralgia may be idiopathic, where the cause is unknown, or due to another underlying medical cause (e.g., multiple sclerosis). It may also occur due to nerve compression by a non-vascular structure, such as a tumor or a cyst.  

Risk factors for the development of trigeminal neuralgia include biological sex, with those born as female more likely to develop the disorder; advanced age, specifically those 50 to 60 years of age; the presence of underlying demyelinating disorders, such as multiple sclerosis; herpes simplex virus infection; acoustic neuroma; saccular aneurysm; and vestibular schwannoma.

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What are the signs and symptoms of trigeminal neuralgia?

The signs and symptoms of trigeminal neuralgia include extreme pain that occurs spontaneously and is exacerbated with touching or moving the tongue, lips, and face. The primary form of trigeminal neuralgia is characterized by severe pain described as intense, sharp, stabbing, and shooting. The individual may report that it feels like a lightning bolt has struck their face or they have suffered an electric shock. The pain is paroxysmal, accompanied by redness and warmth (i.e., flushing), lasts one to several seconds, and is intermittent throughout the day. In between the episodes of sharp pain, the individual may have pain-free periods or dull, constant pain that lasts approximately one to two minutes. In addition to pain, there may be autonomic symptoms, such as lacrimation, conjunctival injection, excessive salivation, and rhinorrhea.  In most cases, the pain and autonomic symptoms are isolated to one side of the face. The pain may worsen with mild tactile stimuli, such as chewing, brushing the teeth, shaving facial hair, blowing one’s nose, and drinking hot or cold drinks. 

The frequency and severity of trigeminal neuralgia can vary among individuals. One individual may have multiple attacks in one day, whereas another may have only one episode every month.  However, the pain tends to worsen with fewer pain-free periods over time. If untreated, trigeminal neuralgia can profoundly affect an individual’s quality of life and has been shown to cause profound psychological effects, such as depression and anxiety.

While trigeminal neuralgia type II is characterized by a less intense pain, typically described as constant, dull, aching, or burning, it can also occur in conjunction with the classic form. Type II trigeminal neuralgia also tends to affect a more widespread portion of the face (e.g., extending across the entire half of the face rather than remaining in the lower jaw). 

How is trigeminal neuralgia diagnosed?

Trigeminal neuralgia is diagnosed using physical examination and obtaining a thorough medical history. This involves assessing for signs of neurological deficit, such as extremity weakness, which can make other diagnoses, such as stroke, more likely. Often, trigeminal neuralgia is referred to as a disease of exclusion; therefore, other potential causes are ruled out before diagnosis. Often, trigeminal neuralgia is referred to as a disease of exclusion; therefore, other potential causes are ruled out before diagnosis. Often, trigeminal neuralgia is referred to as a disease of exclusion; therefore, other possible causes are ruled out before diagnosis. 

Suppose there is no clinical evidence of neurological deficit and no better explanation for facial pain. In that case, two criteria may be used to determine if the individual has trigeminal neuralgia. Criteria A states that the pain occurs in one or more trigeminal nerve divisions (V1-V3), and there is no radiation of pain beyond these divisions. Criteria B says that the pain has three or more of the following four characteristics: recurring paroxysmal attacks less than two minutes in duration; the severe intensity of pain; pain is shock-like, shooting, stabbing, and sharp; and stimulating the affected side of the face results in at least two attacks. To successfully diagnose trigeminal neuralgia, the individual must meet criteria A and B on at least three separate occasions. 

In addition to examination and history-taking, imaging studies may be used to diagnose trigeminal neuralgia. For example, magnetic resonance imaging (MRI) and computed tomography (CT) can identify vasculature or tumors compressing the trigeminal nerve. Another test, called electromyography trigeminal reflex testing, involves using needles to measure the electrical activity of the muscles controlling the nerve and can also be conducted. 

How is trigeminal neuralgia treated?

Trigeminal neuralgia is treated using medications such as carbamazepine, commonly used as an anticonvulsant drug, but has shown efficacy in treating the pain associated with trigeminal neuralgia. It is most effective when used early in the disease course, and most individuals begin with a low dose that gradually increases until the pain dissipates. Other medications with reported efficacy in trigeminal neuralgia include oxcarbazepine, baclofen, and lamotrigine.

Surgical treatments can also provide long-term pain relief, especially if the pain is refractory to medications. Microvascular decompression, also known as the Janetta procedure, can be used to cure trigeminal neuralgia secondary to blood vessel compression. The surgeon will typically make an incision behind the ear on the side of the symptoms, then create a small hole in the skull. The blood vessels pressing on the trigeminal nerve are then moved away, and a pad is placed between the nerve and arteries to prevent future compression. Another possible treatment is stereotactic surgery, which includes procedures known as gamma knife and cyberknife. A concentrated beam of ionizing radiation is used to destroy the trigeminal ganglion or roots, preventing transmission of pain. 

Additional procedures to treat trigeminal neuralgia include percutaneous balloon compression, percutaneous glycerol rhizotomy, and percutaneous stereotactic rhizotomy. All involve the insertion of a needle through the cheek into the trigeminal nerve intending to destroy the nerve. During percutaneous balloon compression, a balloon is inserted and inflated into the trigeminal nerve, damaging the nerve fibers. Percutaneous glycerol rhizotomy involves an injection of glycerol to damage and destroys the nerve. Lastly, percutaneous stereotactic rhizotomy uses an electrode to apply radiofrequency heat waves to damage nerve fibers. 

What are the most important facts to know about trigeminal neuralgia?

Trigeminal neuralgia is a debilitating nerve pain disorder affecting the fifth cranial nerve, the trigeminal nerve. The trigeminal nerve is responsible for supplying sensation to various parts of the face, including the side of the face, the forehead, the lips, and the jaw. There are two types of trigeminal neuralgia, the classic type, the most common, and the secondary type. The classic type is typically caused by vascular compression of the nerve. In contrast, the secondary type is usually caused by compression by a non-vascular cause, such as a tumor or cyst. The diagnosis of trigeminal neuralgia is made on physical examination and patient interview. Other diagnoses are often ruled out, sometimes with the help of diagnostic imaging, to diagnose trigeminal neuralgia. Treatment may include medications, such as carbamazepine, or surgery, including using a gamma knife, percutaneous balloon compression, percutaneous glycerol rhizotomy, and percutaneous stereotactic rhizotomy. 

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Related links

Anatomy of the trigeminal nerve (CN V)
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Resources for research and reference

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