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Disease Profile

Trigeminal 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

US Estimated

Europe Estimated

Age of onset

Adolescent

ICD-10

G50.0

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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Other names (AKA)

Tic Douloureux

Categories

Nervous System Diseases

Summary

Trigeminal neuralgia is a nerve disorder that causes a stabbing or electric-shock-like pain in parts of the face. The pain lasts a few seconds to a few minutes, and usually on only one side of the face. It can also cause muscle spasms in the face the same time as the pain.[1] The pain may result from a blood vessel pressing against the trigeminal nerve (the nerve that carries pain, feeling, and other sensations from the brain to the skin of the face), as a complication of multiple sclerosis, or due to compression of the nerve by a tumor or cyst. In some cases, the cause is unknown.[2] Treatment options include medicines, surgery, and complementary approaches.[3]

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
100% of people have these symptoms
Trigeminal neuralgia
0100661
80%-99% of people have these symptoms
Allodynia
0012533
Cluster headache
0012199
Depressivity
Depression
0000716
30%-79% of people have these symptoms
Difficulty in tongue movements
0000183
Episodic paroxysmal anxiety
0000740
Feeding difficulties
Feeding problems
Poor feeding

[ more ]

 0011968
Mandibular pain
Lower jaw pain
0200025
Paresthesia
Pins and needles feeling
Tingling

[ more ]

 0003401
Poor speech
0002465
Sensory impairment
0003474
Vascular tortuosity
Twisted blood vessels
0004948
5%-29% of people have these symptoms
CNS demyelination
0007305
Cranial nerve compression
0001293
Neoplasm
0002664
Ocular pain
Eye pain
0200026
Peripheral demyelination
0011096
Percent of people who have these symptoms is not available through HPO
Abnormality of the nervous system
Neurologic abnormalities
Neurological abnormality

[ more ]

 0000707
Autosomal dominant inheritance
0000006
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Treatment

Treatment options include medicines, surgery, and complementary approaches.[3]

Anticonvulsant medicines, which are used to block nerve firing, are generally effective in treating trigeminal neuralgia. These drugs include carbamazepine, oxcarbazepine, topiramate, clonazepam, phenytoin, lamotrigine, and valproic acid. Gabapentin or baclofen can be used as a second drug to treat trigeminal neuralgia and may be given in combination with other anticonvulsants.[3]

Tricyclic antidepressants such as amitriptyline or nortriptyline are used to treat pain described as constant, burning, or aching. Typical analgesics and opioids are not usually helpful in treating the sharp, recurring pain caused by trigeminal neuralgia. If medication fails to relieve pain or produces intolerable side effects, surgical treatment may be recommended.[3]

Several neurosurgical procedures are available to treat trigeminal neuralgia. The choice among the various types depends on the nature of the pain, the patient's preference, physical well-being, general health, previous surgeries, presence of multiple sclerosis, and area of trigeminal nerve involvement. Some procedures are done on an outpatient basis, while others may involve a more complex operation that is performed under general anesthesia. Some degree of facial numbness is expected after most of these procedures, and trigeminal neuralgia might return despite the procedure’s initial success. Depending on the procedure, other surgical risks include hearing loss, balance problems, infection, and stroke.[3]

A rhizotomy is a procedure in which select nerve fibers are destroyed to block pain. A rhizotomy for trigeminal neuralgia causes some degree of permanent sensory loss and facial numbness. Several forms of rhizotomy are available to treat trigeminal neuralgia:[3]

  • Balloon compression works by injuring the insulation on nerves that are involved with the sensation of light touch on the face.
  • Glycerol injection involves bathing the ganglion (the central part of the nerve from which the nerve impulses are transmitted) and damaging the insulation of trigeminal nerve fibers.
  • Radiofrequency thermal lesioning involves gradually heating part of the nerve with an electrode, injuring the nerve fibers.
  • Stereotactic radiosurgery uses computer imaging to direct highly focused beams of radiation at the site where the trigeminal nerve exits the brainstem. This causes the slow formation of a lesion on the nerve that disrupts the transmission of pain signals to the brain.
  • Microvascular decompression is the most invasive of all surgeries for trigeminal neuralgia, but it also offers the lowest probability that pain will return. While viewing the trigeminal nerve through a microscope, the surgeon moves away the vessels that are compressing the nerve and places a soft cushion between the nerve and the vessels. Unlike rhizotomies, there is usually no numbness in the face after this surgery. 

A neurectomy, which involves cutting part of the nerve, may be performed during microvascular decompression if no vessel is found to be pressing on the trigeminal nerve.[3]

Some patients choose to manage trigeminal neuralgia using complementary techniques. This is usually done in combination with drug treatment. For some, low-impact exercise, yoga, creative visualization, aromatherapy, or medication helps. Other options include acupuncture, biofeedback, vitamin therapy, nutritional therapy, and electrical stimulation of the nerves.[3]

More detailed information regarding the management of trigeminal neuralgia can be found through the National Institute of Neurological Disorders and Stroke and Medscape.

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

    Social Networking Websites

      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

      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
      • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
          Trigeminal Neuralgia (Neurology)
          Trigeminal Neuralgia Surgery
          Trigeminal Neuralgia in Emergency Medicine
        • 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.
        • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
        • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Trigeminal neuralgia. Click on the link to view a sample search on this topic.

          References

          1. Patient information: Trigeminal neuralgia (The Basics). UpToDate. Waltham, MA: UpToDate; 2016; Accessed 2/17/2016.
          2. Shelat AM. Trigeminal neuralgia. MedlinePlus. May 30, 2016; https://www.nlm.nih.gov/medlineplus/ency/article/000742.htm.
          3. Trigeminal Neuralgia Fact Sheet. National Institute of Neurological Disorders and Stroke (NINDS). 2013; https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Trigeminal-Neuralgia-Fact-Sheet.
          4. Singh MK. Trigeminal Neuralgia: Prognosis. Medscape. November 28, 2016; https://emedicine.medscape.com/article/1145144-overview#a7.

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