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Disease Profile

Migraine with brainstem aura

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

#N/A

ICD-10

#N/A

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)

Bickerstaff migraine; Basilar artery migraine; Brainstem migraine;

Categories

Nervous System Diseases

Summary

Migraine with brainstem aura (MBA) is a type of migraine headache with aura that is associated with pain at the back of the head on both sides. An aura is a group of symptoms that generally serve as a warning sign that a bad headache is coming. Auras may consist of dizziness and vertigo, slurred speech, ataxia, tinnitus, visual changes, and/or loss of balance. Although MBA can occur in men and women of all ages, they are most common in adolescent girls.[1][2] The exact underlying cause of MBA is poorly understood. Migraines are likely complex disorders that are influenced by multiple genes, in combination with lifestyle and environmental factors.[3] In rare cases, susceptibility to MBA may be caused by a change (mutation) in the ATP1A2 gene or CACNA1A gene.[4][5] Treatment during an episode may include nonsteroidal anti-inflammatory drugs (NSAIDs) and antiemetic medications to help ease the symptoms.[2]

Symptoms

Episodes of migraine with brainstem aura (MBA) usually begin with an aura, which is a group of symptoms that serve as a warning sign that a bad headache is coming. Signs and symptoms of an aura vary, but may include:[1][2][6]

  • Dizziness and vertigo
  • Disorientation
  • Double vision and other visual changes
  • Tinnitus
  • Loss of balance
  • Confusion
  • Dysarthria
  • Fainting
  • Loss of consciousness

These symptoms can last any where from two minutes to over an hour. They are then followed by a throbbing headache which is often along the back of the head, and nausea.[2]

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
Percent of people who have these symptoms is not available through HPO
Aphasia
Difficulty finding words
Losing words
Loss of words

[ more ]

0002381
Apraxia
0002186
Autosomal dominant inheritance
0000006
Blurred vision
0000622
Coma
0001259
Confusion
Disorientation
Easily confused
Mental disorientation

[ more ]

0001289
Diplopia
Double vision
0000651
Drowsiness
Sleepy
0002329
Dysarthria
Difficulty articulating speech
0001260
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 with aura
0002077
Seizure
0001250
Transient unilateral blurring of vision
0001125
Vertigo
Dizzy spell
0002321

Cause

The exact underlying cause of migraine with brainstem aura (MBA) is not well understood. MBA, like all types of migraines, are likely complex disorders that are influenced by multiple genes in combination with lifestyle and environmental factors.[3] Scientists also suspect that nerve abnormalities and/or altered blood flow to certain parts of the brain (brainstem and occipital lobes, specifically) may play a role in the development of MBA.[2]

Susceptibility to MBA may rarely be caused by a change (mutation) in the ATP1A2 gene or CACNA1A gene. In these cases, episodes may occur in more than one family member.[4][5]

Diagnosis

A diagnosis of migraine with brainstem aura (MBA) is made based on the presence of characteristic signs and symptoms. Although there are no tests available to confirm the diagnosis, additional testing may be ordered to rule out other conditions that can cause similar symptoms. These tests may include:[2]

Treatment

During an episode of migraine with brainstem aura (MBA), people are usually treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and antiemetic medications to help ease the symptoms. In some cases, a nerve block can be used to treat pain if other therapies are not working. In people with episodes that are frequent, prolonged, or particularly debilitating, medications such as verapamil or topiramate may be prescribed as a preventative therapy.[2]

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

    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.

      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. 
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Migraine with brainstem aura. Click on the link to view a sample search on this topic.

        References

        1. Rima M Dafer, MD, MPH, FAHA. Migraine Variants. Medscape Reference. March 2012; https://emedicine.medscape.com/article/1142731-overview#a1.
        2. David F Black, MD; Carrie Elizabeth Robertson, MD. Migraine with brainstem aura (basilar-type migraine). UpToDate. February 2015; Accessed 2/19/2015.
        3. Basilar-Type Migraine. American Headache Society. 2010; https://www.achenet.org/resources/basilartype_migraine/.
        4. Ambrosini A, D'Onofrio M, Grieco GS, Di Mambro A, Montagna G, Fortini D, Nicoletti F, Nappi G, Sances G, Schoenen J, Buzzi MG, Santorelli FM, Pierelli F. Familial basilar migraine associated with a new mutation in the ATP1A2 gene. Neurology. December 2005; 65(11):1826-1828.
        5. Robbins MS, Lipton RB, Laureta EC, Grosberg BM. CACNA1A nonsense mutation is associated with basilar-type migraine and episodic ataxia type 2. Headache. July 2009; 49(7):1042-1046.
        6. Headache: Hope Through Research. National Institute of Neurological Disorders and Stroke. February 2015; https://www.ninds.nih.gov/disorders/headache/detail_headache.htm.

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