Hemiplegic migraine

Title

Categories:

Congenital and Genetic Diseases; Nervous System Diseases

Subtypes:

Familial hemiplegic migraine

Summary Summary

Hemiplegic migraine (HM) is a rare type of migraine with aura that occurs with motor weakness during the aura. There are two types of HM which are distinguished based on the family history: familial hemiplegic migraine (FHM, in which at least one other family member has the condition) and sporadic hemiplegic migraine (SHM, in which there is no family history).^[1] Signs and symptoms vary but may include visual disturbances, sensory loss, difficulty with speech, weakness on one side of the body, confusion, and impaired consciousness.^[2] Severe attacks may occur in both types with prolonged hemiplegia, coma, fever and/or seizures.^[1] FHM may be caused by a mutation in any of several genes and is inherited in an autosomal dominant manner.^[1]^[2]

Last updated: 11/17/2014

Symptoms Symptoms

The symptoms and severity can vary considerably among people with hemiplegic migraine. Signs and symptoms associated with aura may include:^[3]

Visual disturbance (e.g. blind spots, flashing lights, zigzag pattern, and double vision)
Sensory loss (e.g., numbness or paresthesias of the face or an extremity)
Difficulty with speech (which usually occur along with right-sided weakness)

Motor weakness involves areas affected by sensory symptoms and varies from mild clumsiness to complete deficit. Affected people may also experience neurologic symptoms such as confusion, drowsiness, impaired consciousness, coma, psychosis, and/or memory loss. Neurologic symptoms can last for hours to days. Attention and memory loss can last weeks to months. However, permanent motor, sensory, language, or visual symptoms are extremely rare.^[3]

Last updated: 11/17/2014

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.

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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	Paralysis or weakness of one side of body	0004374
30%-79% of people have these symptoms
Ataxia		0001251
Nystagmus	Involuntary, rapid, rhythmic eye movements	0000639
5%-29% of people have these symptoms
EEG abnormality		0002353
Pigmentary retinopathy		0000580
Sensorineural hearing impairment		0000407
Tremor		0001337
Percent of people who have these symptoms is not available through HPO
Agitation		0000713
Anxiety	Excessive, persistent worry and fear	0000739
Aphasia	Difficulty finding words Losing words Loss of words [ more ]	0002381
Apraxia		0002186
Auditory hallucinations	Hallucinations of sound Hearing sounds [ more ]	0008765
Autosomal dominant inheritance		0000006
Blindness		0000618
Blurred vision		0000622
Cerebellar atrophy	Degeneration of cerebellum	0001272
Coma		0001259
Confusion	Disorientation Easily confused Mental disorientation [ more ]	0001289
Diplopia	Double vision	0000651
Drowsiness	Sleepy	0002329
Dysarthria	Difficulty articulating speech	0001260
Dyscalculia		0002442
Dysphasia		0002357
Episodic ataxia		0002131
Fever		0001945
Hemiparesis	Weakness of one side of body	0001269
Hemiplegia	Paralysis on one side of body	0002301
Incomplete penetrance		0003829
Intellectual disability	Mental deficiency Mental retardation Mental retardation, nonspecific Mental-retardation [ more ]	0001249
Migraine	Intermittent migraine headaches Migraine headache Migraine headaches [ more ]	0002076
Migraine with aura		0002077
Photophobia	Extreme sensitivity of the eyes to light Light hypersensitivity [ more ]	0000613
Psychosis		0000709
Seizures	Seizure	0001250
Transient unilateral blurring of vision		0001125
Vertigo	Dizzy spell	0002321
Visual hallucinations		0002367

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

Last updated: 3/1/2020

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

Cause Cause

There are two types of hemiplegic migraine (HM), which are distinguished based on the family history in affected people: familial hemiplegic migraine (FHM, in which at least one other close family member has the condition) and sporadic hemiplegic migraine (SHM, in which there is no family history).^[1]

FHM may be caused by changes (mutations) in the CACNA1A, ATP1A2, SCN1A, and PRRT2 genes. The first three of these genes give the body instructions for making proteins involved in moving charged atoms (ions) across cell membranes. This process is important for for normal signaling between nerve cells (neurons) in the brain and other parts of the nervous system. Researchers think that mutations in these genes may upset the balance of ions in neurons, affecting the release and uptake of certain neurotransmitters in the brain. The exact function of the PRRT2 gene is not yet understood but is also thought to play a part in controlling signals between neurons. When the genes involved in FHM do not work correctly, the signaling abnormalities between neurons lead to the development of severe headaches and auras in affected people.^[4] The genetic cause in many families with FHM remains unknown.^[3]

While SHM occurs in people with no history of the condition in their family, most cases are due to new (de novo) mutations that occur for the first time in the affected person. Some people with SHM do inherit a mutation from a parent who has the mutation but is not affected. The ATP1A2 and CACNA1A genes have been found to cause SHM in addition to FHM. Many people with SHM do not have a mutation in one of these genes; however, researchers think that mutations in other, unidentified genes are also involved in the condition.^[5]

Last updated: 11/18/2014

Inheritance Inheritance

The known types of familial hemiplegic migraine (FHM) that are due to a mutation in the CACNA1A, ATP1A2, or SCN1A genes are inherited in an autosomal dominant manner.^[6] When a person with an autosomal dominant condition has children, each child has a 50% (1 in 2) risk to inherit the mutated gene from the affected parent. FHM due to a mutation in one of these genes accounts for some, but not all, cases of FHM. It is likely that there are additional types of FHM caused by mutations in other genes.^[6]

People who are the first member of their family to have hemiplegic migraine are classified as having sporadic hemiplegic migraine (SHM). Some cases of SHM are caused by one of the genetic mutations that cause familial hemiplegic migraine (FHM), due to either having a new mutation, or from inheriting the condition from a parent with no signs or symptoms.^[6] In these cases, a person with SHM can still pass the condition on to a child.

Last updated: 11/18/2014

Diagnosis Diagnosis

Hemiplegic migraine is diagnosed based on the presence of specific signs and symptoms. Genetic testing is not necessary for all affected people.^[6]

The diagnosis is based on the presence of having at least 2 attacks with:

aura accompanied by fully reversible motor weakness and fully reversible visual, sensory, and/or speech/language symptoms; and
at least 2 of the following 4 characteristics:
- at least one aura symptom that spreads gradually over ≥5 minutes, and/or two or more symptoms that occur in succession
- each individual non-motor aura symptom lasting 5 to 60 minutes, and motor symptoms lasting <72 hours
- at least one unilateral (one-sided) aura symptom
- the aura being accompanied by headache, or followed by headache within one hour^[6]

Additionally, all other potential causes for the symptoms need to be ruled out (such as transient ischemic attack and stroke). While the criteria requires fully reversible symptoms, it is known that some people with severe attacks may have permanent neurological problems.^[6]

A diagnosis of familial hemiplegic migraine also requires that at least one first or second degree relative has had attacks that meet the above diagnostic criteria. A diagnosis of sporadic hemiplegic migraine requires that no first or second degree relative has had attacks that meet the above criteria.^[6]

Last updated: 11/18/2014

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.

Treatment Treatment

Treatment of hemiplegic migraine varies depending on severity and which symptoms are most problematic for the patient. In general, treatments aim to manage symptoms. Drugs that are effective in the prevention of common migraines may be used in hemiplegic migraine.^[3] Prophylactic management is applied to patients with frequent, long lasting, or severe attacks. Examples of migraine drugs that have been tried with variable success in people with hemiplegic migraine, include oral verapamil, acetazolamide, lamotrigine.^[3]

There are a few articles describing the use of nasal administration of ketamine, intravenous verapamil, and triptans for treatment of aura in people with hemiplegic migraine. Use of triptans in hemiplegic migraine is controversial and may be contraindicated in people with severe attacks.^[3]

For further information on these and other treatments, we recommend that you speak with your healthcare provider.

Last updated: 8/26/2013

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

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

ClinicalTrials.gov lists trials that are related to Hemiplegic migraine. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

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/
Migraine Research Foundation
300 East 75th Street
Suite 3K
New York, NY 10021
Telephone: +1-212-249-5402
Fax: +1-212-249-5405
E-mail: contactmrf@migraineresearchfoundation.org
Website: https://migraineresearchfoundation.org/
National Headache Foundation (NHF)
820 N Orleans, Suite 201
Chicago, IL 60610-3132
Toll-free: 1-888-643-5552
Telephone: +1-312-274-2650
E-mail: info@headaches.org
Website: https://headaches.org
The Migraine Trust
4th Floor
Mitre House
44-46 Fleet Street
London, EC4Y 1BN United Kingdom
Telephone: 0203 9510 150
E-mail: https://www.migrainetrust.org/about-us/contact-us/contact-us/
Website: https://www.migrainetrust.org/

Social Networking Websites

Visit the following Facebook groups related to Hemiplegic migraine:
International Hemiplegic Migraine Foundation (IHMF)
Hemiplegic Migraine Patient Group
Hemiplegic Migraine Caregiver's Support Group
Help With Hemiplegic Migraines

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 Hemiplegic migraine. This website is maintained by the National Library of Medicine.
Sporadic hemiplegic migraine
Familial hemiplegic migraine
The National Institute of Neurological Disorders and Stroke (NINDS) (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
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.
Familial hemiplegic migraine 1
Familial hemiplegic migraine 2
Familial hemiplegic migraine 3
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 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.

Have a question? Contact a GARD Information Specialist.

References References

Anne Ducros. Familial or sporadic hemiplegic migraine. Orphanet. April, 2008; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=569. Accessed 11/17/2014.
Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol. May, 2011; http://www.ncbi.nlm.nih.gov/pubmed/21458376. Accessed 8/26/2013.
Jen JC. Familial Hemiplegic Migraine. GeneReviews. September 2009; http://www.ncbi.nlm.nih.gov/books/NBK1388/#fhm.Clinical_Description.
Familial hemiplegic migraine. Genetics Home Reference. January, 2014; http://ghr.nlm.nih.gov/condition/familial-hemiplegic-migraine. Accessed 12/5/2016.
Sporadic hemiplegic migraine. Genetics Home Reference. February, 2014; http://ghr.nlm.nih.gov/condition/sporadic-hemiplegic-migraine. Accessed 11/18/2014.
Black DF, Robertson CE. Hemiplegic migraine. In: Swanson JW ed.,. UpToDate. Waltham, MA: UpToDate; 2015; https://www.uptodate.com/contents/hemiplegic-migraine.