Migraine with brainstem aura

INTRODUCTION AND DEFINITION — Migraine with brainstem aura (MBA), previously called basilar-type migraine, is an uncommon form of migraine with aura characterized by brainstem symptoms such as dysarthria, vertigo, or ataxia, without evidence of motor weakness.

This topic will review the clinical manifestations, diagnosis, and treatment of MBA. Other aspects of migraine are discussed separately.

●(See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults".)

●(See "Pathophysiology, clinical features, and diagnosis of migraine in children".)

●(See "Acute treatment of migraine in adults".)

●(See "Preventive treatment of episodic migraine in adults".)

●(See "Acute treatment of migraine in children".)

●(See "Preventive treatment of migraine in children".)

TERMINOLOGY — MBA has previously been identified by other terms, reflecting an evolving understanding of the entity [1]. These terms include:

●Bickerstaff migraine

●Basilar migraine

●Basilar artery migraine

●Basilar-type migraine

●Vertebrobasilar migraine

Originally described by Bickerstaff in 1961 as a clinical entity distinct from other forms of migraine [2], earlier descriptions consistent with MBA were given by Aretaeus in ancient Greece and by Gowers in 1907 [2-4]. It was previously considered a disorder of the basilar artery, but MBA is now the preferred term because there is no compelling evidence that the basilar artery is involved in the pathogenesis of the condition. Bickerstaff acknowledged that he had initially "rather loosely termed" this condition basilar artery migraine [5]. In addition, some of the symptoms may localize to brain structures beyond the vascular territory of the basilar artery.

PATHOPHYSIOLOGY — MBA is a subtype of migraine with aura, which is believed to occur when primary neuronal dysfunction leads to increased sensitivity to triggering stimuli. Onset of neuronal dysfunction in the hypothalamus or brainstem may lead to cortical spreading depression, a self-propagating wave of neuronal depolarization and glial activation that spreads across the cerebral cortex. Subsequent stimulation of the trigeminal nerve and neuronal release of vasoactive peptides including substance P, calcitonin gene-related peptide, and serotonin may trigger neurogenic inflammation and pain. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Pathophysiology'.)

However, the pathophysiology of migraine is complex, and the pathogenesis of MBA may be multifactorial. Cortical spreading depression as the cause of altered local blood flow and metabolism in the brainstem has only been proven in animals [6]. Alternative hypotheses suggest that MBA could originate in the cortex [7]. Bickerstaff invoked the vascular hypothesis, the prevailing theory at the time, to explain the symptoms that were referable to either the brainstem or the bilateral occipital lobes [2]. However, ictal spasm in the basilar or posterior cerebral arteries associated with MBA has only been reported in a few cases [8-10]. Despite these few reports, it is unlikely that reversible ischemia is the source of the prolonged symptoms that occur with MBA.

●Distinction from other forms of migraine – The difference between MBA and migraine with typical aura is that the location of the aura symptoms in MBA involves the brainstem or the bilateral occipital hemispheres, whereas in typical migraine the aura symptoms are mainly restricted to a unilateral hemisphere.

●Genetics – Data are limited regarding the genetic basis of MBA. One study reported a mutation in the ATP1A2 gene in three first-degree relatives with MBA [11]. However, no causative mutation for MBA or genetic linkage was identified in a population-based study from Denmark [12].

In another study, a single patient with both MBA and episodic ataxia type 2 had a novel nonsense mutation in the CACNA1A gene, which has been frequently implicated in patients with familial hemiplegic migraine (FHM) [13]. Some authors have suggested FHM may be a genetic form of MBA because nearly 75 percent of patients with hemiplegic migraine exhibit attacks that, if they did not include the additional hemiparesis, would otherwise fulfill diagnostic criteria for MBA [1] (see 'Diagnostic criteria' below). These data suggest a shared pathogenetic mechanism between MBA and hemiplegic migraine and that they may be part of a phenotypic spectrum. (See "Hemiplegic migraine", section on 'Pathophysiology and genetics'.)

EPIDEMIOLOGY — MBA is an uncommon form of migraine, but the actual incidence or prevalence is unknown, as various diagnostic criteria have been applied through the years without uniformity. In a population-based cohort study in Denmark, only 4 cases of MBA (1.37 percent) were found among 293 patients with migraine with aura; based on a reported prevalence of 4 percent for migraine with aura in the general population, the estimated prevalence of MBA may be 0.04 percent [14]. Identifying MBA may also be challenging due to phenotypic overlap with other forms of migraine. In a study of 362 patients with migraine with aura (excluding auras featuring hemiplegia), MBA was identified in 38 patients (10 percent) [12]. Among patients with MBA, preceding attacks of migraine with typical aura occurred in 36 (95 percent).

Initially thought to occur only in adolescent females, MBA may affect patients at any age with a female to male ratio ranging from 1.3:1 to 3.8:1 [12,15,16]. The age of onset is usually between 7 and 20 [12,14,16]. MBA attacks can evolve to more typical common forms of migraine with age.

As with other types of migraine, there is often a family history of migraine among individuals with MBA [2,15,16]. In a series of 30 patients with MBA, a family history of migraine was present in 86 percent [15]. A later age of onset of MBA tended to be associated with a more severe phenotype.

CLINICAL MANIFESTATIONS

Aura — The clinical characteristics of MBA (table 1) were first described in Bickerstaff's 1961 report of 34 patients (26 females), who had attacks of aura lasting 2 to 45 minutes, most often with unilateral or bilateral hemianopic visual disturbance, vertigo, ataxia, dysarthria, bilateral tingling, or numbness [2]. In other studies, the aura in MBA typically lasts from 5 to 60 minutes [12,16]. However, the duration of the MBA aura ranges from several seconds up to 5 days in some patients [12,17,18].

Two or more aura symptoms always occur in attacks of MBA. The frequencies and range of brainstem symptoms were reported in a single center series [12]:

●Vertigo – 61 percent

●Dysarthria – 53 percent

●Tinnitus – 45 percent

●Diplopia – 45 percent

●Bilateral visual symptoms – 40 percent

●Bilateral paresthesias – 24 percent

●Decreased level of consciousness – 24 percent

●Hypacusis – 21 percent

Impairment in or loss of consciousness has been more frequently reported in other studies, accompanying other aura symptoms in as many as 77 percent of patients with MBA [16,19,20]. For such patients, confusion, stupor, or loss of consciousness may last 2 to 30 minutes. Prolonged impairment of consciousness (greater than two weeks) with marked generalized slow-wave delta activity has been reported in one patient with MBA [21].

Migraine triggers in MBA include emotional stress, menstruation, barometric pressure changes, head trauma, specific foods, medications, smoking, and exertion [16].

Headache — Most patients with MBA develop a headache after the aura. In a Danish study of 38 patients with MBA, attacks of MBA were always associated with headache in 28 patients (74 percent), while attacks with or without headache occurred in 10 patients (26 percent) [12].

In the initial description of MBA, headaches were described as throbbing in nature and occipital in location [2]. However, the headache in MBA may localize to other areas of the head, similar to other forms of migraine with aura. The headache is typically severe and may be unilateral or bilateral [20].

Interictal symptoms — Neurologic symptoms with MBA are most commonly transient and resolve after the attack. However, persisting symptoms have been reported in some patients. Specific brainstem localizing syndromes (eg, Wallenberg syndrome) and focal cranial nerve dysfunction have been rarely reported in MBA, perhaps because MBA is a manifestation of a maladaptive central nervous system [22,23].

Some patients with MBA have neuro-otologic abnormalities and may present for medical evaluation for dizziness or other vestibular symptoms. In one series of 50 patients with a history of MBA, most had minor vestibular abnormalities, and a few had severe peripheral injury thought to be a result of basilar migraine [24]. Another series of 20 children with presumed MBA reported that 16 had abnormal bithermal caloric testing [25].

DIAGNOSIS — The diagnosis of MBA is suspected whenever a patient presents with episodic attacks of vertigo, dysarthria, diplopia, ataxia, confusion, or obtundation, particularly when in conjunction with more typical migraine auras consisting of visual, sensory, or aphasic symptoms.

The diagnosis of MBA is made in patients who fulfill the diagnostic criteria, after alternative causes have been excluded. There are no diagnostic tests specific for MBA. (See 'Diagnostic criteria' below and 'Testing to exclude alternative etiologies' below.)

Diagnostic criteria — The diagnostic criteria for MBA from the International Classification of Headache Disorders, 3^rd edition (ICHD-3) are as follows [1]:

●Attacks fulfilling criteria for migraine with aura (table 2)

●Aura with both of the following:

•At least two of the following fully reversible brainstem symptoms:

-Dysarthria

-Vertigo

-Tinnitus

-Hypacusis

-Diplopia

-Ataxia not attributable to sensory deficit

-Decreased level of consciousness (Glasgow Coma Scale (table 3) score ≤13)

•No motor or retinal symptoms

The presence of aura with motor symptoms excludes MBA and points to the diagnosis of hemiplegic migraine [1]. (See "Hemiplegic migraine", section on 'Diagnostic criteria'.)

The presence of an aura characterized by isolated vertigo points instead to a diagnosis of vestibular migraine. (See "Vestibular migraine", section on 'Diagnostic criteria'.)

Testing to exclude alternative etiologies — We suggest neuroimaging for all patients with first-ever MBA symptoms and for those with atypical presentations such as a prolonged aura, minor or absent head pain, abnormal interictal neurologic examination, or when symptoms are refractory to treatment, in agreement with guidelines from the American Headache Society [26]. Other testing is used to exclude specific diagnostic entities in patients with suggestive symptoms.

Repeat neuroimaging and other diagnostic testing are not required for patients with recurrent episodes that are typical for MBA.

●Neuroimaging – We prefer magnetic resonance imaging (MRI) of the brain with contrast and magnetic resonance angiogram (MRA) of the intracranial and extracranial vessels to evaluate for posterior fossa parenchymal or vascular lesions. Examples include brainstem tumor, ischemic stroke or hemorrhage, vertebral or basilar artery thrombosis or dissection, cerebral aneurysms, or arteriovenous malformations. Computed tomography (CT) of the head and CT angiography (CTA) can be performed as an alternative to MRI and MRA or if MRI is contraindicated. Digital subtraction angiography may occasionally be warranted when noninvasive MRA or CTA imaging is nondiagnostic.

Reversible MRI changes may occasionally be seen within the occipital cortex during acute attacks in some patients with MBA [27]. These findings are felt to be due to vasogenic edema and resolve after the attack. However, no specific MRI findings are diagnostic of MBA.

●Electroencephalogram – An electroencephalogram (EEG) is performed in patients with impaired consciousness to exclude seizure activity. In addition, EEG may show transient (nonepileptiform) diffuse slowing associated with an MBA attack in patients with altered consciousness [14]. In one study, slow waves on EEG found during the MBA attack subsequently resolved along with symptoms [16].

●Other testing – A 24-hour cardiac monitor may be used for patients with isolated brainstem symptoms such as dizziness or loss of consciousness to help identify episodic cardiac arrhythmias. Genetic testing is typically reserved for patients with a suggestive family history to evaluate for alternative syndromes such as hemiplegic migraine or episodic ataxia. Vestibular testing and audiometry are warranted for patients with prominent vestibular symptoms or associated hearing loss.

DIFFERENTIAL DIAGNOSIS — The differential diagnosis of MBA includes several conditions that also cause transient brainstem symptoms and signs with or without headache.

●Common entities – The disorders that are most likely to resemble the symptom complex of MBA are transient ischemic attack (TIA) or ischemic stroke involving the brainstem and seizure (table 4) [28].

•TIA or ischemic stroke involving the brainstem, cerebellum, or posterior cerebral artery territory may be caused by thromboembolism, dissection, or atherosclerotic disease of the basilar artery or vertebral arteries. Features specific for cerebrovascular disease include restriction of symptoms to a specific vascular territory and diagnosis by neuroimaging. (See "Posterior circulation cerebrovascular syndromes".)

•Focal seizures featuring brainstem symptoms may occur with or without convulsive activity. They may be preceded by a brief subjective sensory aura (eg, rising epigastric sensation). Other distinguishing features for seizure may be present including head deviation and dystonic limb posturing. EEG may be diagnostic. Ictal or postictal impairment of consciousness with a seizure is common. (See "Focal epilepsy: Causes and clinical features", section on 'Clinical features'.)

●Other conditions – Some primary headache disorders and other conditions characterized by clinical features also found in MBA may be distinguished by the specific pattern to symptoms, the presence of additional signs or symptoms, or by diagnostic testing.

•Otologic or vestibular dysfunction

-Benign paroxysmal positional vertigo is characterized by transient episodes of movement-provoked, isolated vertigo. (See "Benign paroxysmal positional vertigo".)

-Ménière disease is an otologic disorder characterized by recurring episodes of vertigo, hearing loss, and tinnitus. Diagnostic evaluation typically includes audiometry, vestibular testing, and neuroimaging. (See "Meniere disease: Evaluation, diagnosis, and management".)

-Acute labyrinthitis, also called vestibular neuronitis, features vertigo, gait instability, and nystagmus, but headache and other brainstem features are uncommon. (See "Vestibular neuritis and labyrinthitis".)

•Impairment of consciousness

-Encephalopathy induced by a toxic-metabolic state or medication effect may be identified by clinical setting (eg, hospitalized patients), laboratory evaluation, or temporal relationship to medication dosing. (See "Diagnosis of delirium and confusional states".)

-Psychogenic attacks or behavioral spells that feature impairment of consciousness include psychogenic nonepileptic seizures and panic attacks. Other brainstem features are uncommon. (See "Nonepileptic paroxysmal disorders in adolescents and adults", section on 'Psychological disorders'.)

•Ataxia

-Episodic ataxias are inherited conditions characterized by transient episodes of ataxia, nystagmus, and tremor. Episodic ataxias may be identified by genetic testing or a response to treatment with acetazolamide. (See "Overview of the hereditary ataxias", section on 'Episodic ataxias'.)

•Headache

-Familial or sporadic hemiplegic migraine requires the presence of motor weakness as a component of the aura, in contradistinction from MBA. (See "Hemiplegic migraine".)

-Vestibular migraine is a form of migraine with an aura consisting of isolated vestibular symptoms, distinct from MBA that features aura consisting of at least two brainstem symptoms. (See "Vestibular migraine".)

-Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy is a rare syndrome that features migraine, ischemic stroke, and chronic cognitive impairment. (See "Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)".)

-Subarachnoid hemorrhage from a ruptured cerebral aneurysm may present with a thunderclap headache and other symptoms related to the site of bleeding. Diagnosis is by neuroimaging or lumbar puncture. (See "Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis".)

-Tumors of the posterior fossa may present with progressive headaches due to compression of brain structures or hydrocephalus as well as other neurologic symptoms related to the location of the tumor. Diagnosis is typically by neuroimaging. (See "Overview of the clinical features and diagnosis of brain tumors in adults" and "Epidemiology, clinical manifestations, and diagnosis of brain metastases".)

-Chiari I malformation most commonly presents with headache but may also cause other neurologic symptoms including ataxia, cranial neuropathies, and myelopathy. Diagnosis is by identification of cerebellar and brainstem descent on neuroimaging. (See "Chiari malformations".)

-Cerebral venous thrombosis (CVT) most frequently presents with new headache, with or without neurologic signs. Some patients present with focal symptoms or encephalopathy due to elevated intracranial pressure or stroke. Neuroimaging, including with CT or MR venography, is used to identify CVT. (See "Cerebral venous thrombosis: Etiology, clinical features, and diagnosis".)

TREATMENT — The approach to treatment for patients with MBA includes acute therapies to manage active symptoms and preventive therapies for patients with frequent, prolonged, or debilitating attacks. Acute and preventive medication options for patients with MBA are based largely on those used for patients with migraine with aura because available data on treatment of patients with MBA are limited. However, medications that may theoretically cause or exacerbate cerebral ischemia are avoided in patients with MBA. (See 'Medications to avoid' below.)

Acute therapy — We suggest an antiemetic medication or a nonsteroidal anti-inflammatory drug (NSAID) for initial acute symptomatic treatment of patients with MBA. Oral NSAIDs are widely available and inexpensive and may be preferred for patients with milder symptoms. Intravenous (IV) or intramuscular (IM) NSAIDs or antiemetic medications may be preferred for patients with moderate to severe headaches or those that are associated with prominent nausea and vomiting.

For patients with mild to moderate MBA symptoms, options include:

●Ibuprofen 400 to 600 mg orally once

●Naproxen sodium 275 to 750 mg orally once

●Diclofenac 50 to 100 mg orally once

For patients with moderate to severe MBA symptoms or significant nausea/vomiting, options include:

●Ketorolac 30 mg IV or 60 mg IM once (lower doses may be warranted for patients age ≥65 years, <50 kg body weight, or those with kidney impairment)

●Prochlorperazine 10 mg IV, IM, or orally once

●Metoclopramide 10 mg IV or orally once

NSAIDs and antiemetic agents may also be used as combination therapy. Diphenhydramine (25 to 50 mg orally) may be given along with antiemetic medications to prevent akathisia and acute dystonic reactions.

Alternative options for acute symptomatic treatment of MBA include calcitonin gene-related peptide antagonists (eg, rimegepant and ubrogepant) and the serotonin 1F receptor agonist lasmiditan, based on data for and experience with episodic migraine [20]. However, efficacy and safety in patients with MBA has not been studied. Greater occipital nerve blockade using 3 cc of 0.25 percent bupivacaine combined with 40 mg triamcinolone aborted an MBA attack in a single case report [29]. One case of MBA was treated with 0.8 mg of naloxone [9].

Acute migraine treatments are discussed in greater detail separately. (See "Acute treatment of migraine in adults".)

Preventive therapy

First-line options — For patients with MBA who desire preventive therapy because the attacks are frequent, prolonged, or debilitating, we suggest initial treatment with either sustained-release verapamil or topiramate. For adult patients with MBA who fail to benefit with verapamil or topiramate or those who have persistent aura symptoms that predominate over headache, we suggest treatment with lamotrigine.

●Verapamil (sustained-release formulation) is started at 120 mg once daily. The dose can be increased by 120 mg every one to two weeks, based on response and tolerability, up to 360 mg daily given in two to three divided doses. However, we suggest using no more than 120 mg daily for small or older adults and those with hypotension or cardiac conditions. An electrocardiogram should be performed prior to starting verapamil and if cardiac symptoms develop. Adverse effects of verapamil are discussed in greater detail separately. (See "Major side effects and safety of calcium channel blockers".)

●Topiramate is started at 25 mg daily and increased by 25 mg weekly to 100 mg daily as necessary and tolerated or to 200 mg daily in divided doses for patients with a partial response to lower doses. We suggest not using topiramate during pregnancy, due to fetal adverse risks. Topiramate dosing and adverse effects are discussed in greater detail separately. (See "Antiseizure medications: Mechanism of action, pharmacology, and adverse effects", section on 'Topiramate'.)

●Lamotrigine is started at 25 mg daily with slow titration increasing by 25 mg every one to two weeks up to 100 mg per day, based on clinical response and tolerability. Systemic symptoms including rashes and angioedema may occur if dose escalation is too rapid. Lamotrigine should be avoided in patients with cardiac conduction disorders. Lamotrigine dosing and adverse effects are discussed in greater detail separately. (See "Antiseizure medications: Mechanism of action, pharmacology, and adverse effects", section on 'Lamotrigine'.)

Verapamil is used for preventive therapy of MBA largely due to its successful use in patients with hemiplegic migraine [30]. A prospective study of 14 children with MBA reported that prophylaxis with topiramate at both 25 mg daily and 100 mg daily was associated with a significant reduction in overall migraine and MBA frequency [31].

In a case series of 47 patients who had migraine with aura, including six who had MBA, lamotrigine was reported as beneficial in approximately two-thirds [32]. However, an earlier randomized controlled trial did not demonstrate benefit of lamotrigine for migraine prophylaxis in patients with and without aura [33]. Of concern, a benign rash may develop in up to 10 percent of patients during the initial one to two months of lamotrigine therapy and necessitates discontinuation of the drug; the risk of developing a life-threatening rash such as Stevens-Johnson syndrome is approximately 1 in 1000 adults. This risk is higher in children, leading to the recommendation that the drug not be used in patients under the age of 16 years.

Alternative options — Botulinum toxin injections may also be an option for patients with MBA unresponsive to oral agents. In a case series of 11 patients with hemiplegic migraine, onabotulinumtoxinA injections were associated with a reduced frequency and duration of aura in nine patients, including one patient who had symptoms that resembled MBA, with prominent ataxia/dysarthria and presyncope/syncope [34]. Of the 11 patients in this series, 10 met criteria for chronic migraine. Dosing for patients with MBA is based on injection protocols used for patients with chronic migraine. (See "Chronic migraine", section on 'Botulinum toxin'.)

There is meager evidence to support the use of other preventive medications for MBA. Phenytoin and primidone were associated with improvement of MBA in a few patients [35]. Since MBA is a subset of migraine with aura, preventive medications for migraine such as amitriptyline, valproic acid, or calcitonin gene-related peptide antagonists may be beneficial if first-line options are ineffective or intolerable. (See "Preventive treatment of episodic migraine in adults".)

The use of flunarizine for MBA is also based upon its apparent benefit for hemiplegic migraine; flunarizine is not available in the United States. (See "Hemiplegic migraine", section on 'Abortive and preventive medications'.)

Medications to avoid — Some medications used for migraine treatment may lead to cerebral ischemia, based on anecdotal evidence and theoretical concerns [36-38]. These include:

●Triptans

●Ergotamine derivatives

We typically avoid these medications for patients with MBA and frequent or long-lasting auras or auras associated with prominent ataxia or a decreased level of consciousness but may consider their use for selected patients with MBA and auras associated with vertigo, tinnitus, hypacusis, or diplopia.

Triptans and ergotamine derivatives are generally felt to be contraindicated in MBA because of the potential for cerebral vasoconstriction. Some experts have argued that this theoretical risk is based primarily on the relatively outdated vascular theory of migraine, and the contraindication should be reconsidered [39-42]. Small retrospective, observational reports of patients with MBA, hemiplegic migraine, or prolonged aura have shown no serious adverse effects with the use of triptans [40,43] or dihydroergotamine [43,44]. However, historical concerns of worsening vasoconstriction have kept patients with MBA out of larger triptan and dihydroergotamine trials.

Some headache specialists also avoid beta blockers as preventive therapy for patients who have MBA, hemiplegic migraine, or migraine with prolonged aura [45]. The concern is that beta blockers may potentially limit compensatory vasodilatory capacitance of cerebral vessels. Similar to hemiplegic migraine, anecdotal evidence suggests that beta blockers may lead to ischemia in patients with MBA [45,46]. However, multiple case reports have shown an excellent response to treatment with beta blockers for patients with MBA [47].

PROGNOSIS — There are limited data regarding the prognosis of MBA. However, like other forms of migraine, individual attacks of MBA are self-limited and fully reversible in nearly all instances, despite the paroxysmal and alarming nature of the symptoms [2].

Furthermore, the overall prevalence of migraine decreases with age; MBA attacks may remit or evolve to more typical forms of migraine as patients get older. As an example, a retrospective analysis of subjects with onset of migraine with aura in childhood or adolescence identified 13 with MBA [48]. Over a mean follow-up of 11 years, complete remission of headache attacks was noted in five patients, five patients had changed to a different type of headache (eg, migraine without aura or tension-type headache), and MBA persisted in three.

Migrainous infarction may be a rare complication of migraine with aura, though data are limited in MBA. In addition, there is evidence that migraine with aura is associated with a small absolute increase in the overall risk of ischemic stroke. Cerebrovascular complications of migraine are discussed separately. (See "Migraine-associated stroke: risk factors, diagnosis, and prevention".)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Migraine and other primary headache disorders".)

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Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Beyond the Basics topics (see "Patient education: Headache causes and diagnosis in adults (Beyond the Basics)" and "Patient education: Headache treatment in adults (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

●Definition and terminology – Migraine with brainstem aura (MBA), previously called basilar-type migraine, is an uncommon form of migraine with aura with brainstem signs and symptoms but without weakness. (See 'Introduction and definition' above.)

●Clinical features and diagnosis – The diagnosis of MBA includes headache attacks consistent with migraine with aura (table 2) and aura with two or more brainstem-related symptoms (table 1) but excluding motor weakness. (See 'Diagnosis' above.)

Common aura symptoms in MBA include vertigo, dysarthria, tinnitus, visual disturbance, and impairment in consciousness. The aura generally develops over ≥5 minutes, may last up to 60 minutes or longer, and is typically followed by headache. (See 'Clinical manifestations' above.)

●Evaluation for alternative etiologies – We suggest neuroimaging for patients with first-ever MBA symptoms and for those with atypical presentations such as a prolonged aura, minor or absent head pain, abnormal interictal neurologic examination, or when symptoms are refractory to treatment. Repeat neuroimaging and other diagnostic testing is not required for patients with recurrent features typical for MBA. (See 'Testing to exclude alternative etiologies' above.)

●Differential diagnosis – The differential diagnosis of MBA includes several conditions that also cause brainstem symptoms and signs including ischemic stroke, seizure, and other conditions that may also present with brainstem signs (ataxia, vestibular dysfunction, impairment in consciousness) or headache. (See 'Differential diagnosis' above.)

●Management

•Acute treatment – We suggest an antiemetic medication or a nonsteroidal anti-inflammatory drug for acute symptomatic treatment of patients with MBA based on efficacy and tolerability for patients with other forms of migraine (Grade 2C). Alternative options include calcitonin gene-related peptide antagonists (eg, rimegepant and ubrogepant) and lasmiditan. (See 'Acute therapy' above.)

•Initial preventive medications – For patients with MBA who desire preventive therapy because the attacks are frequent, prolonged, or debilitating, we suggest initial treatment with either sustained-release verapamil or topiramate (Grade 2C). (See 'Preventive therapy' above.)

-Verapamil (sustained-release formulation) is started at 120 mg once daily and can be increased by 120 mg based on response and tolerability every one to two weeks up to 360 mg daily given in two to three divided doses. However, we suggest using no more than 120 mg daily for small or older adults and those with hypotension or cardiac conditions.

-Topiramate is started at 25 mg daily and increased by 25 mg weekly to 100 mg daily as necessary and tolerated or to 200 mg daily in divided doses for patients with a partial response to lower doses who are tolerating the medicine well.

Lamotrigine is an alternative treatment for adult patients who do not benefit from or tolerate treatment with verapamil or topiramate and for those who have persistent aura symptoms that predominate over headache. Lamotrigine is started at 25 mg daily with slow titration increasing by 25 mg every one to two weeks up to 100 mg per day, based on clinical response and tolerability.

•Alternative preventive options – Other preventive medications used for migraine may be beneficial for patients with MBA who do not respond to initial preventive therapy. Options include amitriptyline, valproic acid, or calcitonin gene-related peptide antagonists. (See 'Alternative options' above.)

•Medications to avoid – We typically avoid triptans and ergotamine derivatives for patients with MBA and frequent or long-lasting auras or auras associated with prominent ataxia or a decreased level of consciousness due to theoretical concerns and anecdotal evidence that these medications may lead to cerebral ischemia. (See 'Medications to avoid' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges David F Black, MD, who contributed to earlier versions of this topic review.