Rare Nephrology News

Disease Profile

Glossopharyngeal neuralgia

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Adolescent

ICD-10

G52.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Summary

Glossopharyngeal neuralgia is a disorder that is associated with repeated episodes of severe pain in the tongue, throat, ear, and tonsils. These areas are all connected to the ninth cranial nerve, also called the glossopharyngeal nerve. Episodes of pain may last from a few seconds to a few minutes and usually occur on one side of the face. The pain may be triggered by swallowing, speaking, laughing, chewing, or coughing.[1]

Glossopharyngeal neuralgia is thought to be caused by irritation of the glossopharyngeal nerve, but the exact cause of the irritation is sometimes unknown.[1] Laboratory and imaging tests can be used to diagnose the disorder and rule out serious underlying problems. The goal of treatment is to control pain, and the most effective drugs are anti-seizure medications and anti-depressants. Surgery to sever or take pressure off of the glossopharyngeal nerve may be needed in severe cases.[1]

Symptoms

Glossopharyngeal neuralgia causes repeated episodes of severe pain in areas connected to the ninth cranial nerve, also known as the glossopharyngeal nerve. These areas include the back of the nose and throat, back of the tongue, ear, tonsil area, and voice box. Some episodes may also be associated with coughing or hoarseness. Episodes may last for seconds or a few minutes, and they may occur many times throughout the day and night. Episodes may be triggered by coughing, sneezing, swallowing, talking, laughing, or chewing.[1][2][3]

Symptoms of glossopharyngeal neuralgia typically occur in individuals over 40 or 50-years-old.[1][2] Pain usually begins at the back of the tongue or throat, and it sometimes spreads to the ear or the back of the jaw. The pain can cause difficulty speaking and swallowing. In rare cases, the disorder can cause a slow heart rate (bradycardia), hypotension, no heart beat due to cardiac arrest (asystole), or fainting (syncope).[1][2][4]

Cause

Glossopharyngeal neuralgia is thought to be caused by irritation of the glossopharyngeal nerve. In many cases, the source of the irritation is not found.[1][2] In some cases, the source of the irritation is found to be increased pressure on the glossopharyngeal nerve, such as an abnormally positioned artery, growths at the base of the skull, an infection, an injury, or tumors of the throat, mouth, or brain.[1][2] Rarely, the disorder may be caused by an aneurysm in the carotid artery, an abnormally long bone below the ear (styloid process), or multiple sclerosis.[2]

Diagnosis

A diagnosis of glossopharyngeal neuralgia is suspected when a person presents to the doctor with symptoms of the disorder. A test can then be completed in which a cotton swab is touched to the back of the throat. This typically causes pain in people with glossopharyngeal neuralgia, but the pain is relieved when a local anesthetic is applied.[2]

After glossopharyngeal neuralgia is diagnosed, doctors may try to determine the underlying cause of the pain. Tests such as a blood test, CT scan, MRI, or X-rays may be ordered. If it is suspected that there may be problems with the blood vessels, an MR-angiogram (MRA) may also be ordered.[1][2]

Treatment

Treatment for glossopharyngeal neuralgia is aimed at controlling the pain associated with the disorder. Over-the-counter pain medications are generally not very effective at controlling the pain. However, anti-seizure medications and anti-depressants may help relieve pain for some individuals.[1][2] The application of local anesthetics to the affected region may also help control pain, but typically only for a short time.[3] If an underlying cause for the disorder is identified, treatment is generally aimed at treating the underlying problem.[1]

In severe cases, surgery may be necessary to remove pressure from the glossopharyngeal nerve. This may involve removing the cause of the pressure on the nerve or severing the nerve so that it no longer causes pain.[1]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

        In-Depth Information

        • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Glossopharyngeal neuralgia. Click on the link to view a sample search on this topic.

          References

          1. Shelat AM. Glossopharyngeal neuralgia. MedlinePlus. May 30, 2016; https://www.nlm.nih.gov/medlineplus/ency/article/001636.htm.
          2. Rubin M. Glossopharyngeal Neuralgia. Merck Manuals. February 2016; https://www.merckmanuals.com/home/brain-spinal-cord-and-nerve-disorders/cranial-nerve-disorders/glossopharyngeal-neuralgia.
          3. Bajwa ZH, Ho CC, Khan SA, and Garza I. Overview of craniofacial pain. UpToDate. April 25, 2017; https://www.uptodate.com/contents/overview-of-craniofacial-pain.
          4. Krasoudakis A, Anyfantakis D, Hadjipetrou A, et al. Glossopharyngeal neuralgia associated with cardiac syncope: Two case reports and literature review. Int J Surg Case Rep. 2015; 12:4-6. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4485693/.
          5. Glossopharyngeal Neuralgia Information Page. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/Disorders/All-Disorders/Glossopharyngeal-Neuralgia-Information-Page. Accessed 11/12/2017.

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