Glossopharyngeal Neuralgia

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What is Glossopharyngeal Neuralgia

Glossopharyngeal neuralgia is a rare condition in which there are repeated (recurring) attacks of intense pain in the throat, tongue, tonsils, and, sometimes, the ears.

This condition is usually lifelong (chronic), unless successfully treated with surgery.

How common is Glossopharyngeal Neuralgia

The incidence is about 0.2 to 0.7 per 100,000 patients, with most presenting older than 50 years of age and with a female:male ratio of 1:1.

What are the causes?

This condition may be caused by pressure on nerves (glossopharyngeal nerveor vagus nerve) that come from the brain (cranial nerves). Sometimes, the cause is not known (idiopathic glossopharyngeal neuralgia).

What increases the risk?

This condition is more likely to develop in people who are over the age of 40.

What are the symptoms of glossopharyngeal neuralgia

What is the typical clinical presentation of glossopharyngeal neuralgia? 

This syndrome is characterized by paroxysmal jolts or lancinating, sharp pain in the pharynx.

Swallowing is often a trigger.

This pain emanates from a dysfunctional cranial nerve IX (glossopharyngeal nerve).

Some individuals experience syncope from the extreme pain and autonomic dysfunction.

The main symptom of this condition is severe, recurring pain in the ears, tonsils, and the back of the throat and tongue. Pain usually affects only one side of the body (unilateral). The specific location and frequency of the pain varies. You may have times when this condition is inactive (remission), and times when you have pain more frequently. Pain may:

  • Come and go.
  • Last for a few seconds or a few minutes at a time.
  • Happen daily or once every few weeks.

In many cases, certain actions that involve the mouth and throat can trigger pain. These actions are different for each person. Pain can be triggered by:

  • Chewing or swallowing.
  • Talking.
  • Laughing.
  • Yawning.
  • Coughing.
  • Sneezing.
  • Eating spicy foods.

Typically severe stabbing pain along one side of the throat near the tonsillar area with occasional radiation to the ear lasting fractions of a second up to 2 minutes is present, which can be precipitated by swallowing, coughing, talking, or yawning.

Most patients are believed to have an artery compressing the nerve as it exits the medulla and travels through the subarachnoid space to the jugular foramen although secondary causes should be excluded by MRI and other testing (including tumors, multiple sclerosis, Paget’s disease, Sjögren’s syndrome, and other causes). 

How is Glossopharyngeal Neuralgia diagnosed?

This condition is diagnosed based on:

  • The location of your pain.
  • How you feel when you are given medicines that temporarily numb your nerves in a specific area (local anesthetics).
  • Blood tests.
  • Imaging tests, such as:
    • MRI.
    • CT scan.
    • X-rays.

Evaluate the pharynx and hypopharynx by direct visualization to exclude tumor. Head and neck MRI study is the imaging of choice to evaluate the brain stem, palatine region, pharynx, and hypopharynx.

You may be referred to an ear, nose, and throat (ENT) specialist (otolaryngologist) or a health care provider who specializes in the nervous system (neurologist).

How is Glossopharyngeal Neuralgia treated?

Treatment for this condition depends on the cause and severity of your symptoms. Treatment may include:

  • Medicines that decrease nerve irritation, such as medicines to control seizures (anticonvulsants). This is commonly the first treatment method.
  • Medicines that help relieve depression (antidepressants). These may be used to help relieve pain.
  • Local anesthetics. These may be prescribed if other medicines are ineffective.
  • Surgery to relieve pressure on the nerve (vascular decompression) or to cut the nerve (rhizotomy). This may be done if other treatments are ineffective.

The treatment is similar to trigeminal neuralgia. Try oxcarbazepine or carbamazepine as first line.

Other anticonvulsants or baclofen may be tried.

Anesthetic block of the glossopharyngeal nerve is short lived in efficacy.

In recalcitrant cases, microvascular decompression may be pursued. 

Follow these instructions at home:

  • Take over-the-counter and prescription medicines only as told by your health care provider.
  • Do notdrive or operate heavy machinery while taking prescription pain medicine.
  • Keep track of things that trigger your pain. Avoid triggers when possible.
  • Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

  • You have pain that gets worse or does not get better with medicine.
  • You feel light-headed or dizzy.
  • You feel nauseous or you vomit.
  • You have difficulty balancing.

Get help right away if:

  • You have severe pain.
  • You vomit or faint during an attack of pain.
  • Your hearing or vision changes.
  • You lose feeling in your face.
  • You cannot move your face.

Sources

Waldman SD: Atlas of uncommon pain syndromes, 3rd ed. Philadelphia: Elsevier, 2014. Adams H, Pendleton C, Latimer K, et al.: Harvey Cushion’s case series of trigeminal neuralgia, at the John’s Hopkins Hospital: a surgeon’s quest to advance the treatment of “suicide disease.” Acta Neurochir 153(5):1043-1050, 2011. Sheehan J, Pain HC, Strolia M, Steiner L: Gamma knife surgery for trigeminal neuralgia and prognostic factors. J Neurosurg 102(3):434-441, 2005. vanKleef M, van Genderen WE, Narouse S, et al.: Trigeminal neuralgia. Pain Pract 9:252-259, 2009. Benoliel R, Eliav E: Neuropathic orofacial pain. Oral Maxillofac Surg Clin North Am 20:237-254, 2008.

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