Migraine with Brainstem Aura
Posted on June 16 2025,
Migraine with Brainstem Aura
Definition and Terminology
Migraine with brainstem aura (MBA), previously called basilar-type migraine, is an uncommon form of migraine with aura (atypical aura) characterized by symptoms such as vertigo, difficulty speaking, double vision, and hearing changes. Despite its name, emerging evidence suggests these symptoms may not actually originate from the brainstem but rather from cortical (outer brain) areas.
Recent research by Demarquay and colleagues (2018) challenges the traditional view that MBA symptoms come from the brainstem. Their analysis suggests that each "brainstem" symptom can actually originate from specific cortical brain regions. This would explain why MBA and typical migraine aura often occur together and fits better with our understanding of how migraine work through cortical spreading depression.
What This Controversy Means for Patients
Whether your symptoms come from the brainstem or cortical areas doesn't change your diagnosis or treatment, but it does help us understand why MBA often occurs alongside typical migraine aura. The key point is that these symptoms are part of the migraine spectrum and are fully treatable regardless of their exact brain origin.
What is the Brainstem?
The brainstem is the lower part of your brain that connects to the spinal cord. It controls essential functions like breathing, heart rate, blood pressure, and consciousness. It also manages balance, coordination, eye movements, hearing, and speech. This is why the condition is called "migraine with brainstem aura" - because the symptoms relate to functions traditionally controlled by this brain region, whether they actually originate there or from specialized cortical areas that control these same functions.
Evolution of Terminology and Understanding
This condition was first described by Bickerstaff in 1961 as a clinical entity distinct from other forms of migraine. He originally called it "basilar artery migraine" because he believed the symptoms were caused by spasm of the basilar artery. However, Bickerstaff himself later acknowledged that he had "rather loosely termed" this condition basilar artery migraine.
The Name Game: From Blood Vessels to Brain Regions
1961-1988: "Basilar artery migraine" (based on incorrect blood vessel theory)
1988-2004: "Basilar migraine" (removing direct artery reference)
2004-2018: "Basilar-type migraine" (further distancing from vessel theory)
2018-Present: "Migraine with brainstem aura" (recognizing symptom pattern)
Future?: Some researchers now suggest it should be classified as "typical migraine aura" based on cortical origin evidence
Key Distinguishing Features
The main difference between MBA and typical migraine with aura is the type of symptoms experienced. In MBA, patients have at least two symptoms traditionally attributed to brainstem function (like vertigo, double vision, or speech changes), whereas in typical migraine, aura symptoms are mainly visual, sensory, or language-related. However, recent evidence suggests this distinction may be less clear-cut than previously thought, as these symptom patterns may all originate from different cortical regions.
Epidemiology and Who Gets It
MBA is an uncommon form of migraine, but the actual incidence is unknown because different diagnostic criteria have been used over the years. Recent population studies suggest it affects about 0.04% of the general population, making it quite rare.
Demographics and Risk Factors
Initially thought to occur only in adolescent females, we now know MBA can affect patients at any age, with most cases beginning before age 50. There's often a strong family history of migraine, and many patients with MBA also experience typical migraine attacks.
Genetic Factors
Data on the genetic basis of MBA are limited. Some studies have found mutations in genes like ATP1A2 and CACNA1A in families with MBA, but no single causative gene has been identified. Some researchers suggest MBA may be part of a spectrum that includes other genetic migraine conditions like familial hemiplegic migraine.
Family Connection
If you have MBA, there's a good chance other family members have migraine too. While we haven't found a single "MBA gene," the strong family clustering suggests genetics play an important role. This doesn't mean your children will definitely get MBA, but they may have a higher chance of developing some form of migraine.
How MBA Happens in the Brain: The Brainstem vs. Cortical Debate
The exact mechanism behind MBA is hotly debated in neuroscience. While traditionally thought to involve the brainstem, groundbreaking research suggests MBA symptoms may actually originate from specific cortical (outer brain) regions through the same cortical spreading depression mechanism seen in typical migraine aura.
The Cortical Origin Theory (Demarquay et al., 2018)
Recent research proposes that each "brainstem" symptom can originate from specific cortical areas: vertigo from vestibular cortex dysfunction, hearing changes and tinnitus from auditory cortex problems, double vision from parieto-occipital involvement, speech difficulties from precentral gyri dysfunction, balance problems from parietal lobe processing issues, and consciousness changes from prefrontal and posterior parietal cortices. This theory explains why typical and brainstem aura often occur together.
What is Cortical Spreading Depression?
Think of cortical spreading depression like a slow wave moving across your brain, temporarily changing how nerve cells work. It's like when you drop a stone in a pond and see ripples spread outward, except this happens with brain electrical activity. Whether this wave affects brainstem areas directly or specialized cortical regions that control balance, speech, and vision, you get the symptoms of MBA.
Competing Theories
The Evidence for Cortical Origin
A landmark 2018 study by Demarquay and colleagues reviewed clinical observations and brain mapping data that support cortical origins for MBA symptoms. They found that vertigo can result from vestibular cortex dysfunction, tinnitus and hearing changes from auditory cortex problems, double vision from parieto-occipital involvement, speech difficulties from precentral gyri dysfunction, balance problems from parietal lobe processing issues, and consciousness changes from specific cortical networks.
Why This Matters
The cortical origin theory has important implications:
- Explains why 95% of MBA patients also have typical migraine aura
- Supports the idea that MBA should be classified as typical migraine aura
- Fits better with cortical spreading depression mechanisms
- May influence future treatment approaches and research directions
- Challenges outdated restrictions on certain migraine medications
Neurochemical Mechanisms
MBA involves the release of various brain chemicals including calcitonin gene-related peptide (CGRP), substance P, and serotonin. These chemicals can affect blood vessels and nerve activity in brainstem regions or specialized cortical areas, leading to the characteristic symptoms. This is why newer migraine medications that target these chemicals can be effective for MBA regardless of whether symptoms originate from brainstem or cortical regions.
Symptoms and Clinical Features
MBA is characterized by specific brainstem-related symptoms that occur during the aura phase, typically followed by headache. The key requirement is having at least two brainstem symptoms during an attack, and these symptoms must be fully reversible.
Required Brainstem Symptoms
Understanding MBA Symptoms
Vertigo: A spinning sensation, different from lightheadedness
Dysarthria: Slurred or unclear speech, difficulty pronouncing words
Diplopia: Seeing double images
Tinnitus: Ringing, buzzing, or other sounds in the ears
Hypacusis: Temporary hearing loss or muffled hearing
Ataxia: Loss of coordination, unsteadiness when walking
Timing and Duration
The aura in MBA typically lasts from 5 to 60 minutes, though it can range from several seconds up to 5 days in some patients. The aura generally develops gradually over at least 5 minutes, and symptoms may occur one after another. Most patients develop a headache after the aura, though the headache may be milder than their usual migraine attacks.
Consciousness Changes
One of the more concerning symptoms of MBA is impairment of consciousness, which can occur in up to 77% of patients. This may include confusion, stupor, or even loss of consciousness lasting 2 to 30 minutes. Rarely, consciousness problems can persist for 24 hours or longer, though this is extremely uncommon.
While MBA episodes are typically harmless, you should seek immediate medical attention if you experience loss of consciousness for the first time, symptoms that are much more severe than usual, symptoms lasting longer than typical, or any new neurological symptoms you haven't had before. These could indicate a more serious condition that needs immediate evaluation.
Associated Features
Along with brainstem symptoms, patients may experience typical migraine aura features like visual disturbances (zigzag lines, blind spots), sensory changes (numbness, tingling), or speech problems. The headache that follows is typically severe and may be on one or both sides of the head, often described as throbbing.
Common Triggers
MBA triggers are similar to those for other migraine subtypes and include emotional stress, menstruation, changes in barometric pressure, head trauma, and smoking.
Diagnostic Criteria for Migraine with Brainstem Aura
International diagnostic criteria from the International Classification of Headache Disorders (ICHD-3) for definitive diagnosis of MBA.
Requirement | Specific Criteria | Clinical Notes |
---|---|---|
Basic Migraine Criteria |
Must fulfill criteria for migraine with aura
|
Standard migraine with aura requirements must be met first |
Brainstem Symptoms |
≥2 of the following fully reversible symptoms:
|
At least two brainstem symptoms required; symptoms must completely resolve |
Exclusion Criteria |
No motor or retinal symptoms
|
Presence of weakness points to hemiplegic migraine instead |
Alternative Diagnoses | Not better explained by another disorder | Stroke, seizure, and other brainstem disorders must be ruled out |
Differential Diagnosis
Several conditions can mimic MBA, making accurate diagnosis challenging. The most important alternatives to consider are stroke, seizure, and other vestibular disorders. Careful history-taking and sometimes additional testing help distinguish MBA from these conditions.
Most Important Conditions to Rule Out
Stroke vs. MBA
Brainstem stroke typically has sudden onset and symptoms that persist or worsen, while MBA symptoms develop gradually and completely resolve. Stroke patients often have vascular risk factors like high blood pressure, diabetes, or heart disease. Emergency imaging (MRI or CT) can help distinguish between the two conditions.
When Emergency Evaluation is Needed
Seek immediate medical care if you have sudden onset of brainstem symptoms (rather than gradual development), symptoms that don't resolve, persistent weakness or numbness, severe headache unlike previous ones, or if you have risk factors for stroke like heart disease, high blood pressure, or diabetes. When in doubt, it's always better to get evaluated promptly.
Other Vestibular Conditions
Vestibular migraine causes isolated episodes of vertigo with migraine features but doesn't include other brainstem symptoms. Meniere disease causes vertigo with hearing loss, tinnitus, and ear fullness. Benign paroxysmal positional vertigo (BPPV) causes brief episodes of vertigo triggered by specific head movements.
Required Testing
First-time MBA symptoms require neuroimaging (preferably MRI with contrast) to rule out structural problems like stroke, tumors, or blood vessel abnormalities. EEG may be needed if there's concern about seizures. Patients with typical recurrent episodes don't need repeat testing.
Imaging Recommendations
Brain MRI with contrast and magnetic resonance angiography (MRA) is preferred to evaluate for:
- Brainstem tumor or other masses
- Stroke or brain hemorrhage
- Blood vessel problems (aneurysms, malformations)
- Vertebral or basilar artery disease
Acute Treatment for MBA Episodes
Evidence-based acute treatment options for MBA episodes, with important considerations about medications to avoid due to theoretical stroke risk.
Drug Class | Medications & Dosing | Safety Profile | Clinical Notes |
---|---|---|---|
First-Line Options |
NSAIDs: • Ibuprofen 400-600 mg • Naproxen 275-750 mg • Diclofenac 50-100 mg Antiemetics: • Prochlorperazine 10 mg • Metoclopramide 10 mg |
Safe for MBA Well-tolerated, widely available |
Preferred for initial treatment; IV/IM forms available for severe symptoms |
Alternative Options |
CGRP Antagonists: • Rimegepant • Ubrogepant Lasmiditan (Serotonin 1F agonist) |
Safe for MBA Newer agents, limited data in MBA specifically |
May be effective based on general migraine data; require further study in MBA |
Generally Avoided |
Triptans: • Sumatriptan • Rizatriptan • Others Ergotamines: • DHE • Ergotamine |
Theoretical stroke risk May cause blood vessel constriction |
Avoided due to concern about worsening brainstem blood flow; some experts debate this restriction |
Supportive Care |
Environment: • Dark, quiet room • Rest • Hydration Severe cases: • IV fluids • Hospital observation |
Always safe No medication risks |
Important for all patients; hospitalization may be needed for consciousness changes |
Preventive Treatment
Preventive therapy should be considered for patients with frequent episodes (typically more than 2-4 per month), prolonged attacks, or episodes that significantly impact daily life. Treatment options are based largely on medications used for other forms of migraine, with some specific considerations for MBA.
First-Line Preventive Options
Understanding Preventive Medications
Verapamil: A calcium channel blocker originally used for heart conditions. Shown to be effective for MBA in studies. Requires heart monitoring.
Topiramate: An anti-seizure medication that also prevents migraine. Can cause weight loss and cognitive changes in some people.
Lamotrigine: Another anti-seizure medication particularly good for preventing aura symptoms. Requires slow dose increases to avoid rash.
Dosing and Monitoring
Verapamil: Start at 120 mg once daily, increase by 120 mg every 1-2 weeks up to 360 mg daily in divided doses. Requires baseline EKG and monitoring for heart rhythm changes. Use lower doses in elderly or those with heart conditions.
Topiramate: Start at 25 mg daily, increase by 25 mg weekly to 100 mg daily as needed, up to 200 mg in divided doses for partial response. Avoid in pregnancy due to birth defect risk.
Lamotrigine: Start at 25 mg daily, increase by 25 mg every 1-2 weeks up to 100 mg daily. Slow titration is essential to prevent serious rash (Stevens-Johnson syndrome). Not recommended under age 16.
Alternative Preventive Options
Second-Line Treatments
If first-line options fail or aren't tolerated, consider:
- Botulinum toxin injections: May help reduce aura frequency and duration
- Standard migraine preventives: Amitriptyline, valproic acid, or CGRP antagonists
- Flunarizine: Effective for hemiplegic migraine, may help MBA (not available in US)
Medications with Concerns
Some headache specialists avoid beta blockers in MBA patients due to theoretical concerns about limiting the brain's ability to compensate for reduced blood flow. However, multiple case reports show excellent response to beta blockers, and the evidence for harm is limited. The decision should be individualized based on patient factors and response to other treatments.
Special Considerations for MBA Prevention
MBA prevention focuses on reducing both aura frequency and severity. Since many MBA patients also have typical migraine attacks, successful prevention often helps both types of episodes. Treatment should be maintained for at least 6-12 months before considering discontinuation, and lifestyle modifications (sleep hygiene, stress management, etc.) remain important adjuncts to medication.
Prognosis and Long-term Outlook
The long-term outlook for MBA is generally good, with individual attacks being self-limited and fully reversible in nearly all cases. Like other forms of migraine, MBA tends to improve with age, and many patients see their attacks change to more typical migraine patterns over time.
Natural History
A retrospective study following 13 patients with childhood-onset MBA over an average of 11 years found that 5 patients had complete remission of headaches, 5 changed to different headache patterns (like migraine without aura or tension headaches), and only 3 continued to have MBA. This suggests that MBA often evolves or improves over time.
Stroke Risk Considerations
While individual MBA attacks are harmless, there is evidence that migraine with aura (including MBA) is associated with a small increase in stroke risk. This risk is primarily relevant for women who smoke or use estrogen-containing birth control pills. The absolute risk remains very low, but these risk factors should be discussed with healthcare providers.
What This Means for You
If you have MBA, the most important things to know are: (1) Individual attacks, while frightening, are not dangerous and will resolve completely, (2) The condition often improves with age or changes to less severe forms, (3) Effective preventive treatments are available if attacks are frequent, and (4) While there's a small increased stroke risk, this can be minimized by avoiding smoking and discussing birth control options with your doctor.
Factors for Better Outcomes
Optimizing Long-term Prognosis
Several factors can improve outcomes for MBA patients:
- Early accurate diagnosis and patient education
- Identification and avoidance of personal triggers
- Appropriate preventive treatment when indicated
- Regular follow-up with healthcare providers familiar with MBA
- Lifestyle modifications (stress management, sleep hygiene, regular exercise)
- Avoiding additional stroke risk factors (smoking, certain medications)
Rare Complications
While extremely rare, migrainous infarction (stroke during a migraine attack) has been reported. This is more likely in patients with additional stroke risk factors. The overall risk remains very low, but patients should be educated about when to seek emergency care for atypical or prolonged symptoms.
"While migraine with brainstem aura symptoms can be alarming, understanding the condition leads to appropriate treatment and reassurance for patients and families." - Cerebral Torque
This information is for educational purposes only and should not replace professional medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider for personalized medical care.
References
- Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1):1-211.
- Bickerstaff ER. Basilar artery migraine. Lancet. 1961;1(7169):15-17.
- Kirchmann M, Thomsen LL, Olesen J. Basilar-type migraine: clinical, epidemiologic, and genetic features. Neurology. 2006;66(6):880-886.
- Yamani N, Chalmer MA, Olesen J. Migraine with brainstem aura: defining the core syndrome. Brain. 2019;142(12):3868-3878.
- Lapkin ML, Golden GS. Basilar artery migraine. A review of 30 cases. Am J Dis Child. 1978;132(3):278-281.
- Sturzenegger MH, Meienberg O. Basilar artery migraine: a follow-up study of 82 cases. Headache. 1985;25(8):408-415.
- Evans RW, Linder SL. Management of basilar migraine. Headache. 2002;42(5):383-386.
- Lewis D, Paradiso E. A double-blind, dose comparison study of topiramate for prophylaxis of basilar-type migraine in children: a pilot study. Headache. 2007;47(10):1409-1417.
- Pascual J, Caminero AB, Mateos V, et al. Preventing disturbing migraine aura with lamotrigine: an open study. Headache. 2004;44(10):1024-1028.
- Baron EP, Tepper SJ, Mays M, Cherian N. Acute treatment of basilar-type migraine with greater occipital nerve blockade. Headache. 2010;50(6):1057-1059.
- Chen TY, Garza I, Dodick DW, Robertson CE. The Effect of OnabotulinumtoxinA on Aura Frequency and Severity in Patients With Hemiplegic Migraine: Case Series of 11 Patients. Headache. 2018;58(7):973-981.
- Termine C, Ferri M, Livetti G, et al. Migraine with aura with onset in childhood and adolescence: long-term natural history and prognostic factors. Cephalalgia. 2010;30(6):674-681.
- Ambrosini A, D'Onofrio M, Grieco GS, et al. Familial basilar migraine associated with a new mutation in the ATP1A2 gene. Neurology. 2005;65(11):1826-1828.
- Robbins MS, Lipton RB, Laureta EC, Grosberg BM. CACNA1A nonsense mutation is associated with basilar-type migraine and episodic ataxia type 2. Headache. 2009;49(7):1042-1047.
- Evans RW, Burch RC, Frishberg BM, et al. Neuroimaging for Migraine: The American Headache Society Systematic Review and Evidence-Based Guideline. Headache. 2020;60(2):318-336.
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