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.
Glossopharyngeal neuralgia (GPN) is a unilateral severe pain felt in the distribution of the glossopharyngeal nerve, the auricular (Jacobsen’s nerve) and pharyngeal branches of the vagus nerve. Pain occurs in the ear, the posterior third of the tongue, and the tonsillar fossa.
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.
GPN is a rare disorder, 1/100 as common as trigeminal neuralgia. It occurs most frequently in middle-aged men and women.
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).
The majority of GPN cases are idiopathic or are related to neurovascular compression of cranial nerves IX and X. Tumors involving the jugular foramen can cause GPN as well.
Eagle syndrome is another uncommon but well-described cause, where an elongated styloid process or ossified stylohyoid ligament compresses the glossopharyngeal nerve.
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.
- 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).
Pain is elicited by stimulating trigger points in the cutaneous distribution of the glossopharyngeal or vagus nerves, often with swallowing, chewing, yawning, or coughing. Note that while most triggers mimic those of trigeminal neuralgia, swallowing is specific to GPN. In “classic” GPN, patients are pain-free in between severe episodes that are brief and lancinating. In “symptomatic” GPN, an aching pain is present between the episodes of stabbing pain. Episodes often occur in clusters, and patients tend to sit leaning forward and drool during attacks. Syncope occurs in 10% of cases, due to hypersensitivity of the dorsal motor nucleus of the vagus nerve.
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.
tests, such as:
- CT scan.
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).
GPN is a clinical diagnosis. Imaging investigations can include magnetic resonance imaging (MRI) to assess for vascular compression and to rule out neoplastic lesions and demyelinating disease. Computed tomography or plain films are also recommended to assess for Eagle syndrome. The diagnosis can often be confirmed by the cessation of pain with a nerve block at the jugular foramen or with application of topical anesthesia to the pharynx.
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.
What are the surgical options for treatment of glossopharyngeal neuralgia?
Surgical treatment is reserved for cases of drug inefficacy or intolerance. Microvascular decompression of the cranial nerves IX and X is first line, often producing excellent results. If no vascular compression is present, sectioning of the nerve IX and upper rootlets of nerve X can be performed. Other options include percutaneous radiofrequency rhizotomy and radiosurgery. In the event of Eagle syndrome, the pain can be cured by resection of the styloid process.
Antiepileptic medications such as carbamazepine and gabapentin are commonly used to treat GPN. If syncope is a prominent feature of the disease, atropine can also be used.
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.
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.