Migraine with brainstem aura

Alternate names

Bickerstaff migraine; Basilar artery migraine; Brainstem migraine; Vertebrobasilar migraine; Basilar migraine; Basilar-type migraine; MBA

Definition

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.

Epidemiology

Actual prevalence and incidence are unknown. It is more common in females than in males. It can occur at all ages but is more common in adolescence and young adults. The age of onset is usually 7 to 20.

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.
• 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.
• 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.

Inheritance

Autosomal dominant pattern, a 50/50 chance.

• In most cases, migraines with brainstem aura (MBA) are not inherited.
• However, in rare cases, susceptibility to MBA may be caused by a change (mutation) in the ATP1A2 gene or CACNA1A gene.
• In these cases, an increased risk for MBA is inherited in an autosomal dominant manner.
• This means that to be affected, a person only needs a mutation in one copy of the responsible gene in each cell. In some cases, an affected person inherits the mutation from an affected parent. Other cases may result from new (de novo) mutations in the gene. These cases occur in people with no history of the disorder in their family. A person with one of these mutations has a 50% chance with each pregnancy of passing along the altered gene to his or her child.

Signs and 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:

• 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.

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:

• Brain MRI
• MR angiogram (MRA)
• Electroencephalogram
• 24-hour heart monitor
• Specialized blood tests

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.

Prognosis

• Due to the rarity of these migraines, there is limited data on the long-term outlook (prognosis) for affected people.
• Although the symptoms are often severe during episodes, they generally resolve on their own with no lasting complications.
• Some research suggests that the frequency of MBA decreases with age, although they may evolve into episodes of more typical forms of migraines in some affected people.
• Migraines with aura do carry a small risk of migrainous infarction (stroke in the setting of a migraine attack) and ischemic stroke.

NIH genetic and rare disease info

• NIH info on Migraine with brainstem aura

Migraine with brainstem aura is a rare disease.

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