Cranial Neuralgia, Types, Causes, Diagnosis and Treatment

Cranial neuralgia is pain associated with the nerves of the head that provide sensation and control movement in the face, scalp, neck and throat.

Pain can arise in any of these areas, depending on the cranial nerves affected. Certain senses, such as taste, smell, vision or hearing, may become impaired due to damage or inflammation of the cranial nerves.

There are 12 sets of cranial nerves. Different types of cranial neuralgia may occur, depending on the nerves affected. For example, trigeminal neuralgia affects the largest nerve in the skull (trigeminal nerve) and is the most common type of cranial neuralgia. It causes sharp bursts of pain along one side of the face that may be triggered by touch.

Glossopharyngeal neuralgia involves the glossopharyngeal and vagus nerves. This type of cranial neuralgia can affect the tongue, throat, neck and ears. Additional types of cranial neuralgia include occipital neuralgia, laryngeal neuralgia and nervus intermedius neuralgia.

Lyme disease is an infection caused by a deer tick bite that can lead to problems if untreated.Cranial neuralgia can have a variety of causes, including nerve compression and physical trauma. Certain conditions or infections may also cause cranial neuralgia, including diabetes, multiple sclerosis and Lyme disease. In some cases, the cause of cranial neuralgia remains unknown.

The primary symptom of cranial neuralgia is recurrent pain in the same area of the head. The severity of pain can vary greatly. The pain may fade, but is likely to return and it often occurs along the length of the affected cranial nerve. Depending on the type of neuralgia involved, the pain may be described in many ways, including sharp, burning or shock-like. In addition, some patients may also experience itching, numbness and muscle weakness.

To diagnose a patient’s cranial neuralgia, a physician must first rule out other possible causes of head or facial pain. This is likely to require blood tests and a neurological examination. The patient’s medical history will also be reviewed to identify potential triggers of pain. The patient may also require a dental examination to eliminate possible oral disorders that could be causing facial pain.

Treatment of cranial neuralgia is often only necessary when the pain becomes debilitating. When it is necessary, there are several treatment options possible. Many analgesic medications, including antidepressants and anticonvulsants, may be used to reduce the pain of patients with cranial neuralgia. In addition, surgery may help by removing structures (e.g., tumors, lesions) that are compressing the nerve. In most cases, one treatment or a combination of treatments is successful.

Neuralgia is the sensation of pain along a nerve or nerve pathway. Cranial neuralgia is this type of pain associated with the nerves of the head.

Twelve pairs of cranial nerves connect the base of the brain to the sense organs and muscles of the head. The nerves pass through small openings (foramina) in the skull, with one of each pair going to the right and the other going to the left side of the head.

The cranial nerves are part of the peripheral nervous system. They do not process information like the brain and spinal cord (central nervous system). Instead, they carry signals from the senses (sensory neurons) and transmit commands to the muscles and glands (motor neurons).

Cranial nerves are identified by name and Roman numeral. For example, the largest nerve in the skull (trigeminal nerve) is also known as the fifth cranial nerve. They may be sensory neurons, motor neurons or a mix. The 12 pairs of cranial nerves are:

I. Olfactory. Sensory neuron related to smell.

II. Optic. Sensory neuron related to vision.

III. Oculomotor. Motor neuron involved in movement of the eyelids and eyes.

IV. Trochlear. Motor neuron involved in eye movement.

V. Trigeminal. Mixed function. Sensory function is related to facial skin and its motor function controls chewing.

VI. Abducent. Motor neuron involved in eye movement.

VII. Facial. Mixed function. Sensory neuron related to taste as well as motor function for facial expression and mouth movement.

VIII. Vestibulocochlear (also known as the auditory or acoustic nerves). Sensory neuron related to hearing and balance.

IX. Glossopharyngeal. Mixed function. Senses taste and controls glands in mouth and swallowing muscles.

X. Vagus. Mixed function. Sensory function is related to skin of and near the ears and motor control of glands in the membranes of chest and abdomen organs (e.g., heart, lungs, intestines).

XI. Accessory. Motor neuron involved in swallowing and head and shoulder movement.

XII. Hypoglossal. Motor neuron involved in tongue movement.

Most cases of cranial neuralgia are related to damage to the trigeminal nerve, but any of the cranial nerves may be affected, such as the glossopharyngeal or vagus nerves. This damage may be linked to compression, infection or inflammation of the nerves, but in many cases there are no apparent physical changes causing the pain.

Anatomy of the spine includes the cervical spine, thoracic spine, lumbar spine and sacral region. Neuralgia may also affect the occipital nerve, which provides sensory information from the back of the head but is not a cranial nerve. Instead it exits the spinal cord, near where the spine meets the skull. Cranial neuralgia can involve any of these nerves and the resulting pain is often felt in the face, scalp, neck, throat or inside the mouth.

Cranial neuralgia is not typically life-threatening. It may cause mild pain that passes without treatment. However, some patients experience incapacitating pain that severely limits their ability to function. In addition, some cases of cranial neuralgia may result from a potentially fatal condition (e.g., brain tumor) that requires medical attention. It is recommended that patients who experience recurrent head or facial pain see their physician for diagnosis and potential treatment.

Types of cranial neuralgias

There are many types of cranial neuralgia. They differ depending on the cranial nerves involved and the patterns of pain that result. The types of cranial neuralgia include:

Trigeminal neuralgia. The most common type of cranial neuralgia, according to the National Institutes of Health. The trigeminal (fifth cranial) nerve is the largest of the cranial nerves, making this type of neuralgia one of the most debilitating.
Patients often feel pain in sharp bursts (usually less than two minutes’ duration) that occur along only one side of the face. Pain is usually in the lower face or jaw (and not often in the temple or forehead area). It may be triggered by touching the skin of the face, or when chewing, talking, swallowing or brushing the teeth.

The condition may also be referred to as tic douloureux because of the wincing reaction to facial pain that many patients demonstrate. Cases tend to occur most frequently in patients who are 50 years of age or older.

Glossopharyngeal neuralgia.

This is an uncommon type of cranial neuralgia that primarily involves the glossopharyngeal (ninth cranial) nerve. This nerve runs into the tongue and the throat.
Patients may feel electric shock-like pain that usually is in the back of the throat and back of the tongue. The pain may be triggered by chewing, sneezing, swallowing, speaking, coughing, yawning, spicy foods, or contact with the neck or ear. It occurs most often in men after the age of 40. In some cases, glossopharyngeal neuralgia may lower the patient’s heart rate and lead to fainting.

Vagal and superior laryngeal neuralgia.

This involves the vagus (10th cranial) nerve. The pain is similar to glossopharyngeal neuralgia, and the two conditions can occur together. It begins in the throat and radiates to the ear or eye. In severe cases, the pain may prevent speech. It may be triggered by coughing, swallowing or talking.

Nervus intermedius neuralgia (also known as geniculate neuralgia). Pain due to this form of neuralgia is described as a cutting or shocking sensation deep within the ear. It occurs in patterns similar to that of glossopharyngeal neuralgia and some physicians believe they may actually be the same condition. It may also be associated with the seventh cranial nerve (facial nerve).

Raeder syndrome. Like trigeminal neuralgia, Raeder syndrome also causes pain along the fifth cranial nerve (trigeminal nerve). It occurs less frequently than trigeminal neuralgia and is most likely in middle-aged or older male patients. They experience a constant burning sensation near one eye that fades after a few weeks or months.

Cluster-tic syndrome. A combination of trigeminal neuralgia and cluster headaches. Cluster headaches are a group of headaches located near the eye that occur over several months. Patients tend to be between 20 and 70 years old and may experience chronic pain or recurrences and remissions.
In addition, postherpetic neuralgia, the most common type of neuralgia, can damage the cranial nerves (Herpes zoster oticus) as well as other nerves throughout the body. Postherpetic neuralgia is caused by the virus responsible for chickenpox and shingles (varicella-zoster virus). Unlike most forms of cranial neuralgia, herpes zoster oticus may affect multiple nerves. Patients experience ear pain and a rash on the head or neck. When associated with facial paralysis, the condition is known as Ramsay Hunt syndrome.

Occipital neuralgia is also a source of head pain, but it is not caused by damage to a cranial nerve. Instead, it involves the occipital nerve. This cervical nerve leaves the top of the spinal cord and runs up to the back of the head. Damage to this nerve results in pain described as aching, pressure, stabbing, throbbing or shooting. It begins in the back of the head near the neck and often spreads to the forehead and scalp. Because it causes head pain, some medical groups, including the International Headache Society, classify it as a type of cranial neuralgia.

Risk factors and causes of cranial neuralgias
Cranial neuralgia (pain associated with cranial nerves) can have a variety of causes, though they may not be identifiable. In some cases, the cranial nerves become damaged due to nerve compression. The nerves can become compressed or pinched by nearby structures, such as blood vessels or tumors. They can also be damaged by toxic chemicals, medications or physical trauma (including surgery), which can lead to cranial neuralgia. In some cases, the cause of a patient’s cranial neuralgia cannot be determined.

Certain conditions or infections can also cause cranial neuralgia. Diabetes damages the blood vessels that supply nerves, which can cause nerve malfunction and nerve loss. Other kidney and blood disorders (e.g., porphyria) allow toxins to accumulate in the blood, which can lead to neuralgia.

Osteoarthritis is the most common type of arthritis and is caused by joint cartilage deterioration.Other conditions that may also be related to cranial neuralgia include multiple sclerosis, arthritis, syphilis and Lyme disease. Herpes zoster oticus and postherpetic neuralgia may also result from nerve damage caused by the virus responsible for chickenpox and shingles.

Cranial neuralgia may occur in anyone, but is more likely in certain populations. Many forms are more likely to occur in older patients. For example, glossopharyngeal neuralgia is more common in patients older than 40, and trigeminal neuralgia is more common in those older than 50. There are also some types (e.g., Raeder syndrome) that occur more often or only in men. In contrast, trigeminal neuralgia is more likely to occur in women. In some instances, trigeminal neuralgia occurs in families, which may be an indication of a genetic link.

Signs and symptoms of cranial neuralgias
The primary symptom of cranial neuralgia is recurring pain that affects certain areas of the head. It usually recurs in the same location, and is often felt near the surface of the skin or scalp. The pain is likely to move over parts of the head and follows the path of the damaged nerve. Sometimes ear pain or eye pain is involved. In many cases, certain pain triggers can be identified, which may help patients avoid those triggers.

The types of cranial neuralgia and the associated head or facial pain include:

Trigeminal neuralgia. Sharp bursts of pain along only one side of the face, especially in the lower face or jaw.

Glossopharyngeal neuralgia. Electric shock-like pain that originates in the throat and radiates to the ears.

Vagal and superior laryngeal neuralgia. Pain that primarily affects the throat and jaw.

Nervus intermedius neuralgia. A cutting or shocking sensation deep within the ear.

Herpes zoster oticus. Intense pain in and around the ear.

Raeder syndrome. Constant burning sensation near one eye.

Cluster-tic syndrome. A combination of trigeminal neuralgia and cluster headaches located near the eye.
Pain related to other forms of headaches tends to affect broader areas of the head than those related to cranial neuralgia. In addition, some forms of cranial neuralgia may be characterized by cycles of pain remission and recurrence. In progressive conditions, the periods of remission may become shorter and less frequent as the disease advances. For other symptoms specific to certain types of cranial neuralgias, see Types and differences.

Other signs and symptoms related to cranial neuralgia may include:

Sensitivity to touch and temperature change
Itching and numbness
Muscle weakness or paralysis
Lack of sweating
Abnormal skin sensations
Sensory loss

Diagnosis methods for cranial neuralgias
A physician should be consulted if cranial neuralgia is suspected. Before a diagnosis can be made, a physician will likely review the patient’s medical history and perform a neurological examination. The medical history will include questions about a patient’s symptoms. Factors common to multiple occurrences of symptoms may indicate triggering mechanisms. The patient may be asked to complete a pain assessment form.

The neurological examination typically tests the patient’s reflexes, coordination and mental status. During this examination, a physician will look for any tenderness along the pathways of cranial nerves and whether any nerve dysfunction is present, such as abnormal reflex reactions.

To confirm a diagnosis of cranial neuralgia, the physician must first rule out other possible causes of head pain, such as migraines or other headaches, temporal arteritis, infections, fractures or arthritis. This is likely to require additional analyses, including blood tests. These tests can identify potential causes of cranial neuralgia, such as high blood sugar and kidney malfunction.

An electromyogram (EMG) and nerve conduction velocity study (NCS) test may also be performed to assess whether symptoms are the result of damage to the muscle or nerves, and the extent of damage. The EMG measures the health of muscle tissue, and the NCV test indicates the effectiveness of certain nerves. In some cases, a patient may be asked to undergo a dental examination to determine whether oral health factors are responsible for the patient’s pain.

Electrodiagnostics assess muscle function (e.g., electromyography [EMG], nerve conduction study). MRI is an imaging test used in pain diagnosis, to guide treatment and to monitor for relapse.
Imaging tests may also be performed. This may include an MRI (magnetic resonance imaging) of the brain, which can indicate whether tumors or other structures are compressing a cranial nerve and causing a patient’s cranial neuralgia. MRIs may also help confirm nerve disorders that can cause cranial neuralgia, such as multiple sclerosis. In those cases, a spinal tap may also be performed.

Treatment and prevention of cranial neuralgias
In some cases of cranial neuralgia, the pain fades with time and without any treatment. In other cases, treatment is necessary to reduce a patient’s symptoms. The type of treatment used depends on the cause of a patient’s cranial neuralgia, the severity and location of the pain.

There are many medications available to help reduce the pain of cranial neuralgia. Treatment may begin with recommendations for over-the-counter analgesics. If they do not work, the physician may prescribe stronger pain medications, including:

Antidepressants. These medications are involved in regulating the neurotransmitters of the brain. They are typically used to reduce the symptoms of depression, but they also work to reduce pain associated with cranial neuralgia.

Anticonvulsants. Medications that reduce the electrical activity in the brain. They also help reduce pain due to cranial neuralgia.
In most cases, patients and physicians will need to work together to monitor the patient’s response to the medication. It may take some time to find the proper medication and dosage to treat the patient’s symptoms. In some cases, the medication will not cure the cranial neuralgia and may not reduce the pain. In these instances, other treatments may be necessary. They may include:

Rest. The simplest treatment may be reducing physical stress and allowing the body to respond to the cause of the pain.

Injection therapy. Local anesthetics may be injected into the area near the damaged nerve to reduce pain.

Physical therapy. Exercises may be recommended by a physical therapist that can help reduce the occurrence of pain related to cranial neuralgia.

Massage therapy. Manipulation of the skin and tendons around the damaged nerves may help reduce the compression responsible for the patient’s pain.

Surgery. In some cases, treatment may require removal of a tumor or adjusting the position of a blood vessel that is compressing the nerve. In other cases, all or part of the nerve may be removed to reduce the pain. This often works, but patients may also lose other sensations related to the nerve.
In most cases, cranial neuralgia occurs without warning and cannot be prevented. One exception is herpes zoster oticus, which is sometimes prevented with early treatment of shingles. Proper personal care in relation to other conditions (e.g., diabetic patients monitoring their glucose levels) can help prevent associated cases of cranial neuralgia. In some patients, keeping a diary of cranial neuralgia attacks (e.g., when, where, environmental or other factors) may help identify triggers that may be avoided in the future.

Questions for your doctor on cranial neuralgias

Preparing questions in advance can help patients have more meaningful discussions with healthcare professionals regarding their condition. Patients may wish to ask their doctor the following questions about cranial neuralgias:

  1. Why do you think I have cranial neuralgia?
  2. What type of cranial neuralgia do I have?
  3. What could have caused my cranial neuralgia?
  4. Are there any tests you will need to perform to confirm your diagnosis, or to identify the cause of my cranial neuralgia? How do I prepare for these tests?
  5. Will my pain get worse over time?
  6. Do my symptoms indicate a more serious disease?
  7. Is my condition hereditary?
  8. What are my treatment options? What do you recommend, and what risks or side effects are involved?
  9. For how long will I need this treatment?
  10. What is the likelihood my symptoms will return after treatment?

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