Familial hemiplegic migraine

Title

Other Names:

FHM; Hemiplegic Migraine, Familial; Hemiplegic-ophthalmoplegic migraine

Categories:

Congenital and Genetic Diseases; Nervous System Diseases

Subtypes:

Familial hemiplegic migraine type 1; Familial hemiplegic migraine type 2; Familial hemiplegic migraine type 3

This disease is grouped under:

Hemiplegic migraine

Summary Summary

Familial hemiplegic migraine (FHM) is a form of migraine headache that runs in families. Migraines usually cause intense, throbbing pain in one area of the head, often accompanied by nausea, vomiting, and extreme sensitivity to light and sound. These recurrent headaches typically begin in childhood or adolescence and may last from a few hours to a few days. People with familial hemiplegic migraine experience an aura that comes before the headache. The most common symptoms associated with an aura are temporary visual changes such as blind spots (scotomas), flashing lights, zig-zagging lines, and double vision. In people with familial hemiplegic migraine, auras are also characterized by temporary numbness or weakness, often affecting one side of the body (hemiparesis). An aura typically develops gradually over a few minutes and lasts about an hour. Researchers have identified three forms of familial hemiplegic migraine known as FHM1, FHM2, and FHM3. Each of the three types is caused by mutations in a different gene. Treatment varies depending on signs and symptoms and may include use of anti migraine or anti seizure medications.^[1]

Last updated: 11/28/2016

Symptoms 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.

Showing of 8 |

Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Abnormality of movement	Movement disorder Unusual movement [ more ]	0100022
Hemiplegia/hemiparesis		0004374
30%-79% of people have these symptoms
Ataxia		0001251
Nystagmus	Involuntary, rapid, rhythmic eye movements	0000639
5%-29% of people have these symptoms
Dysphasia		0002357
EEG abnormality		0002353
Pigmentary retinopathy		0000580
Sensorineural hearing impairment		0000407

Showing of 8 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 9/1/2018

Do you have updated information on this disease? We want to hear from you.

Diagnosis Diagnosis

Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

Testing Resources

The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.
Orphanet lists international laboratories offering diagnostic testing for this condition.

Treatment Treatment

There is no cure for FHM and no standardized treatment recommendations. Various migraine medications (tricyclic antidepressants, beta blockers, calcium channel blockers) and anti seizure medications (topiramate, lacosamide, levetiracetam, valproate) may be utilized for individuals with frequent attacks. In general, medications that constrict the blood vessels (vasoconstricting agents) should be avoided as they may increase the risk of stroke.^[1]

Last updated: 11/28/2016

Management Guidelines

Orphanet Emergency Guidelines is an article which is expert-authored and peer-reviewed that is intended to guide health care professionals in emergency situations involving this condition.

Prognosis Prognosis

Although most people with FHM recover completely between episodes, neurological symptoms such as memory loss and problems with attention can last for weeks or months. The occurrence of FHM attacks tends to decrease with age. The eventual neurologic outcome is often benign; however, about 20 percent of individuals with FHM develop mild but permanent difficulty coordinating movements (ataxia), which may worsen with time, and nystagmus (rapid, involuntary eye movements). Unusually severe migraine episodes have been reported in some people with FHM. These episodes may include fever, seizures, prolonged weakness, coma, and, rarely, death.^[2]^[1]

Last updated: 11/28/2016

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition.

Organizations 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

American Migraine Foundation (AMF)
19 Mantua Rd.
Mount Royal, NJ 08061
Telephone: 856-423-0043
Fax: 856-423-0082
E-mail: amf@talley.com
Website: https://americanmigrainefoundation.org/
The Migraine Trust
52-53 Russell Square
London, WC1B 4HP United Kingdom
Telephone: 020 7631 6970
Fax: 020 7436 2886
E-mail: info@migrainetrust.org
Website: http://www.migrainetrust.org/

Social Networking Websites

Visit the following Facebook groups or pages related to Familial hemiplegic migraine:
International Hemiplegic Migraine Foundation (IHMF)
Hemiplegic Migraine Patient Group
Hemiplegic Migraine Caregiver's Support Group

Do you know of an organization? We want to hear from you.

Learn More 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

Genetics Home Reference (GHR) contains information on Familial hemiplegic migraine. This website is maintained by the National Library of Medicine.
The Migraine Trust, a support organization, offers information on Familial hemiplegic migraine

In-Depth Information

GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions.
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.
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 Familial hemiplegic migraine. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

What is the prognosis for memory retention when patient is on prophylactic medications such a Topamax? The migraines are interfering with my work, yet the Topamax makes me fuzzy as well. Will that resolve? See answer

Have a question? Contact a GARD Information Specialist.

References References

Jen JC. Familial hemiplegic migraine. GeneReviews. May 14, 2015; https://www.ncbi.nlm.nih.gov/books/NBK1388/. Accessed 11/28/2016.
Familial hemiplegic migraine. Genetics Home Reference. January, 2014; http://ghr.nlm.nih.gov/condition/familial-hemiplegic-migraine. Accessed 12/5/2016.

Do you know of a review article? We want to hear from you.

You can help advance
rare disease research! You can help advance rare disease research!