Skip to main content
U.S. Department of Health & Human Services
National Institutes of Health
NCATS
National Center for Advancing and Translational Sciences National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences
1-888-205-2311
Menu Search
1-888-205-2311
Menu
  • Home
  • Diseases expand submenu for Diseases
    • Browse A-Z
    • Find Diseases By Category expand submenu for Find Diseases By Category
      • Autoimmune / Autoinflammatory diseases
      • Bacterial infections
      • Behavioral and mental disorders
      • Blood Diseases
      • Chromosome Disorders
      • Congenital and Genetic Diseases
      • Connective tissue diseases
      • Digestive Diseases
      • Ear, Nose, and Throat Diseases
      • Endocrine Diseases
      • Environmental Diseases
      • Eye diseases
      • Female Reproductive Diseases
      • Fungal infections
      • Heart Diseases
      • Hereditary Cancer Syndromes
      • Immune System Diseases
      • Kidney and Urinary Diseases
      • Lung Diseases
      • Male Reproductive Diseases
      • Metabolic disorders
      • Mouth Diseases
      • Musculoskeletal Diseases
      • Myelodysplastic Syndromes
      • Nervous System Diseases
      • Newborn Screening
      • Nutritional diseases
      • Parasitic diseases
      • Rare Cancers
      • RDCRN
      • Skin Diseases
      • Viral infections
    • List of FDA Orphan Drugs
    • GARD Information Navigator
    • FAQs About Rare Diseases
  • Guides expand submenu for Guides
    • Patients, Families and Friends expand submenu for Patients, Families and Friends
      • How to Find a Disease Specialist
      • Tips for the Undiagnosed
      • Support for Patients and Families
      • Tips for Finding Financial Aid
      • Help with Travel Costs
      • How to Get Involved in Research
      • FAQs About Chromosome Disorders
      • Medical and Science Glossaries
    • Healthcare Professionals expand submenu for Healthcare Professionals
      • Caring for Your Patient with a Rare Disease
      • ICD Coding for Rare Diseases
    • Researchers expand submenu for Researchers
      • Finding Funding Opportunities
    • Teachers and Students expand submenu for Teachers and Students
      • Teaching Resources
      • K's Korner
  • News expand submenu for News
    • In The Spotlight
    • News Archive
    • Connect with GARD
    • Media Requests
  • About GARD expand submenu for About GARD
    • Contact GARD
    • About GARD
    • Videos
    • Brochures and Publications
  • En Español expand submenu for En Español
    • Enfermedades
    • Contacte GARD
    • Guías de Información expand submenu for Guías de Información
      • Preguntas Más Frecuentes Sobre Enfermedades Raras
      • Como Encontrar un Especialista en su Enfermedad
      • Consejos Para una Condición no Diagnosticada
      • Ayuda para Pacientes y Familias
      • Ayuda Financiera para Mi Enfermedad
      • Participe en Estudios de Investigación
      • Preguntas Más Frecuentes Sobre los Trastornos Cromosómicos
    • Sobre GARD
  • Home
© Positive Exposure
banner showing people with disabilities and/or their relatives
  1. Home
  2. Diseases
  3. Hemiplegic migraine
print
  • Table of Contents expand submenu for Table of Contents
    • Summary
    • Symptoms
    • Cause
    • Inheritance
    • Diagnosis
    • Treatment
    • Find a Specialist
    • Research
    • Organizations
    • Learn More
    • News & Events
    • Related Diseases
    • GARD Answers
    • References
  • Browse A-Z
  • Find Diseases By Category expand submenu for Find Diseases By Category
    • Autoimmune / Autoinflammatory diseases
    • Bacterial infections
    • Behavioral and mental disorders
    • Blood Diseases
    • Chromosome Disorders
    • Congenital and Genetic Diseases
    • Connective tissue diseases
    • Digestive Diseases
    • Ear, Nose, and Throat Diseases
    • Endocrine Diseases
    • Environmental Diseases
    • Eye diseases
    • Female Reproductive Diseases
    • Fungal infections
    • Heart Diseases
    • Hereditary Cancer Syndromes
    • Immune System Diseases
    • Kidney and Urinary Diseases
    • Lung Diseases
    • Male Reproductive Diseases
    • Metabolic disorders
    • Mouth Diseases
    • Musculoskeletal Diseases
    • Myelodysplastic Syndromes
    • Nervous System Diseases
    • Newborn Screening
    • Nutritional diseases
    • Parasitic diseases
    • Rare Cancers
    • RDCRN
    • Skin Diseases
    • Viral infections
  • List of FDA Orphan Drugs
  • GARD Information Navigator
  • FAQs About Rare Diseases
You can help advance
rare disease research!
You can help advance rare disease research!
toolbox
Find out how with the NCATS Toolkit.

Hemiplegic migraine


Title


Categories:
Congenital and Genetic Diseases; Nervous System Diseases

Summary Summary


Listen
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


Listen
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.

Showing of 37 |
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 0000639
5%-29% of people have these symptoms
EEG abnormality 0002353
Pigmentary retinopathy 0000580
Seizures
Seizure
0001250
Sensorineural hearing impairment 0000407
Tremor
Tremors
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 0001269
Hemiplegia 0002301
Incomplete penetrance 0003829
Intellectual disability
Mental deficiency
Mental retardation
Mental retardation, nonspecific
Mental-retardation
[ more ]
0001249
Migraine with aura 0002077
Photophobia
Extreme sensitivity of the eyes to light
Light hypersensitivity
[ more ]
0000613
Psychosis 0000709
Transient unilateral blurring of vision 0001125
Vertigo
Dizzy spell
0002321
Visual hallucinations 0002367
Showing of 37 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 4/1/2018

Cause Cause


Listen
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


Listen
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


Listen
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

Genetic testing for familial hemiplegic migraine (FHM) is available in order to try to establish the genetic basis of the condition in a family.[3] However, the genes currently known to cause FHM account for only a small proportion of cases,[6] so most affected people will not have a positive genetic test result. Prenatal diagnosis and testing for at-risk family members is available if the disease-causing mutation in a family is already known.[3]

The Genetic Testing Registry (GTR) provides information about the labs that offer genetic testing. 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.
Last updated: 11/18/2014

Treatment Treatment


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

Find a Specialist Find a Specialist


Listen

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


Listen

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


Listen

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: 212-249-5402
    Fax: 212-249-5405
    E-mail: contactmrf@migraineresearchfoundation.org
    Website: http://www.migraineresearchfoundation.org
  • National Headache Foundation
    820 North Orleans
    Chicago, IL 60610-3132
    Toll-free: 888-643-5552
    E-mail: info@headaches.org
    Website: http://www.headaches.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 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


Listen

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.

News & Events News & Events


Listen

News

  • National DNA Day Reddit "Ask Me Anything" (AMA) Series
    April 11, 2018

Related Diseases Related Diseases


Listen

The following diseases are related to Hemiplegic migraine. If you have a question about any of these diseases, you can contact GARD.

  • Familial hemiplegic migraine

GARD Answers GARD Answers


Listen

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

  • My husband has been having issues for about 3 years now. His left side extremities go numb/tingly. It's debilitating. No headache but does have auras. He's had numerous tests, including MRIs and a spinal tap. We've been told possible MS and saw a local doctor as well as a neurologist at a major medical center. We were told it was 'nothing' and to live with it. It seems the barometric pressure really triggers his symptoms. Would weather changes cause symptoms like this? We have no answers but would welcome any theories. See answer

  • I am curious how accurate hemiplegic migraine testing is. Standardly they test for 3 genes. Familial Hemiplegic Migraine and Sporadic. If I'm correct it is CACNA1A, ATP1A2 and SCN1A. Does everyone with hemiplegic migraine test positive for these mutations and if not, do you know the percentage? See answer

  • I experienced a headache followed by left sided numbness. I believe my doctor said I had hemiplegic migraine. During these episodes the left side of my body will feel like tiny pinpricks and go completely numb. When it gets really bad it spreads over to my right side and i cannot operate my car. When this happens I do not always get headaches. Is there any information you can give me? See answer


Have a question? Contact a GARD Information Specialist.

References References


  1. 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.
  2. 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.
  3. Jen JC. Familial Hemiplegic Migraine. GeneReviews. September 2009; http://www.ncbi.nlm.nih.gov/books/NBK1388/#fhm.Clinical_Description.
  4. Familial hemiplegic migraine. Genetics Home Reference. January, 2014; http://ghr.nlm.nih.gov/condition/familial-hemiplegic-migraine. Accessed 12/5/2016.
  5. Sporadic hemiplegic migraine. Genetics Home Reference. February, 2014; http://ghr.nlm.nih.gov/condition/sporadic-hemiplegic-migraine. Accessed 11/18/2014.
  6. Black DF, Robertson CE. Hemiplegic migraine. In: Swanson JW ed.,. UpToDate. Waltham, MA: UpToDate; 2015; https://www.uptodate.com/contents/hemiplegic-migraine.
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!
toolbox
Find out how with the NCATS Toolkit.

Share this content:

Share this content:

twitter-icon facebook-icon contact-icon link-icons

Copy Link

Link copied to your clipboard.

printer-icon

You May Be Interested In

How to Find a Disease Specialist

How to Find a Disease Specialist

Tips for the Undiagnosed

Tips for the Undiagnosed

Support for Patients and Families

Support for Patients and Families

Tips for Finding Financial Aid

Tips for Finding Financial Aid

Help with Travel Costs

Help with Travel Costs

How to Get Involved in Research

How to Get Involved in Research

FAQs About Chromosome Disorders

FAQs About Chromosome Disorders

Medical and Science Glossaries

Medical and Science Glossaries

Caring for Your Patient with a Rare Disease

Caring for Your Patient with a Rare Disease

ICD Coding for Rare Diseases

ICD Coding for Rare Diseases

Finding Funding Opportunities

Finding Funding Opportunities

Teaching Resources

Teaching Resources

K's Korner

K's Korner

placeholder for the horizontal scroll slider Back to top
GARD Home NCATS Home Site Map Browse Glossary A-Z Privacy Notice Disclaimer Accessibility FOIA OIG

If you have problems viewing PDF files, download the latest version of Adobe Reader

For language access assistance, contact the NCATS Public Information Officer

Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 - Toll-free: 1-888-205-2311

contact gard Office of Rare Disease Research Facebook Page Office of Rare Disease Research on Twitter
Listen