Migraine with Brainstem Aura. 2024
Migraine with Brainstem Aura
Bickerstaff, in his seminal article entitled, “Basilar Artery Migraine” published in The Lancet in 1961 began with the following statement from Aretaeus, a Greek physician from Cappadocia circ. A.D. 100:
“If darkness possesses the eyes, and if the head be whirled round with dizziness, and the ears ring as from the sound of rivers rolling along with a great noise, or like the wind when it roars among the sails, or like the clang of pipes or reeds, or like the rattling of a carriage, we call the affection Scotoma (or vertigo). The mode of vertigo is heaviness of the head, sparkles of light in the eyes along with much darkness, ignorance of themselves and those around, and if the disease go on increasing, the limbs sink below them and they crawl on the ground; there is nausea and vomiting of phlegm, or of yellow or black bilious matter.”
How did this syndrome which had originally been named, “Basilar Artery Migraine,” change into Bickerstaff migraine, basilar migraine; and basilar-type migraine end up being called Migraine with Brainstem Aura?
How is Migraine with Brainstem Aura different from migraine with aura?
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This is an article by Britt Talley Daniel MD, retired member of the American Academy of Neurology, the American Headache Society, migraine textbook author, podcaster, YouTube video producer, and blogger.
Migraine with Brainstem Aura is a type of migraine with aura attack with symptoms from the brainstem causing headache on the back of the head and visual auras and numbness and tingling on both sides of the body, left and right.
Specific definition of Migraine with brainstem aura.
According to the International Classification of Headache Disorders V 3 (ICHD 3) Migraine with brainstem aura (MBA) has the following features.
Migraine with Brainstem Aura: It occurs with neurological symptoms that would include at least two of the following:
slurring of speech (dysarthria)
a sensation of movement (vertigo)
ringing in the ears (tinnitus)
double vision (diplopia)
Unsteadiness when walking as if drunk (ataxia)
Temporary decreased consciousness (syncope)
Pins and needles and /or numbness affecting both arms and/or legs
Changes in eyesight in both eyes such as patterns or flashing lights
Migraine with brainstem aura symptoms often develop gradually and occur with or before a typical migraine headache in those who experience it.
Migraine with brainstem aura occurs in about 1 in 10 people who get migraine with typical visual aura. Vertigo, dizziness, slurred speech, ringing in the ears and double vision would also commonly occur.
Some people experience disorientation or confusion as well as temporary loss of consciousness, known as syncope.
In general, most people with migraine with brainstem aura first experience symptoms in adult life although it can occur at any age. However, if a first migraine attack develops after 50, it will need further investigation such as an MRI scan, to rule out some other causes.
People who experience migraine with brainstem aura also experience migraine with typical aura symptoms.
Migraine with brainstem aura is described as: migraine with aura symptoms clearly originating from the brainstem, but with no motor weakness.
Diagnostic criteria:
Attacks fulfilling criteria for Migraine with aura and Migraine with Brainstem aura.
Aura with both of the following:
at least two of the following fully reversible brainstem symptoms:
a) dysarthria (slurred speech)
b) vertigo (spinning sensation)
c) tinnitus (ringing in the ears)
d) hypoacusis (decreased hearing volume)
e) diplopia (double vision)
f) ataxia not attributable to sensory deficit (off balance)
g) decreased level of consciousness (GCS ≤13)5
no motor 6 or retinal symptoms.
Notes:
Dysarthria should be distinguished from aphasia.
Vertigo does not embrace and should be distinguished from dizziness.
This criterion is not fulfilled by sensations of ear fullness.
Diplopia does not embrace (or exclude) blurred vision.
The Glasgow Coma Scale (GCS) score may have been assessed during admission; alternatively, deficits clearly described by the patient allow GCS estimation.
When motor symptoms are present, code as 1.2.3 Hemiplegic migraine.
Comments:
Originally the terms basilar artery migraine or basilar migraine were used but, since involvement of the basilar artery is unlikely, the term migraine with brainstem aura is preferred.
There are typical aura symptoms in addition to the brainstem symptoms during most attacks. Many patients who have attacks with brainstem aura also report other attacks with typical aura and should be coded for both 1.2.1 Migraine with typical aura and 1.2.2 Migraine with brainstem aura.
Many of the symptoms listed may occur with anxiety and hyperventilation and are therefore subject to misinterpretation.
Nosology
Basilar-type migraine, basilar migraine, basilar artery migraine, and Bickerstaff migraine are now referred to as migraine with brainstem aura. Migraine with aura symptoms clearly originate from the brainstem and are not ischemic in etiology.
Because involvement of the basilar artery is unlikely, the term migraine with brainstem aura is preferred.
Pathophysiology
Bickerstaff, writing in 1961, invoked the vascular hypothesis, the prevailing theory at the time, to explain the symptoms of "basilar artery migraine" that were referable to either the brainstem or the bioccipital hemispheres.
In a later publication, he acknowledged that he had "rather loosely termed" this condition basilar artery migraine.
There is no evidence that the basilar artery is involved in the etiology of MBA, and abnormal flow in the basilar artery has never been proven in MBA.
Two cases, one with familial hemiplegic migraine with MBA-like symptoms, and one with MBA, have shown ictal spasm of the basilar artery on angiography.
Another reported case showed reduced mean flow velocity in both posterior cerebral arteries during a single MBA episode with resolution after the aura.
Despite these reports, it is unlikely that reversible ischemia is the source of the prolonged symptoms that occur with MBA.
Cortical spreading depression (CSD) is believed to be the neuronal mechanism that generates Migraine with brainstem aura symptoms similar to the typical visual auras that occur in migraine.
The current hypothesis is that cortical spreading depression occurs either in the brainstem or simultaneously and bilaterally in the cerebral cortex.
Clinical description
The attack usually starts with aura symptoms which may be decreased hearing ability (hypoacusis), dysarthria (slurred speech), loss of consciousness (syncope or fainting), vertigo (spinning), ataxia (off balance), tinnitus (a ringing or sound in the ear), or diplopia (double vision).
After the aura the pain of migraine with brainstem aura (MBA) usually locates bilaterally in the occipital portion of the head. With worsening headache, nausea and vomiting may occur.
Vertigo, slurred speech, tinnitus and diplopia are the commonest reported symptoms.
Some individuals experience disorientation or confusion in addition to transient loss of consciousness termed syncope.
Most individuals with MBA first experience symptoms between late adolescence and their 20s.
Frequency
Migraine with brainstem aura is a rare episodic disorder and occurs in 1.5% of patients with headache. MBA occurs in about 10% of individuals who get migraine with typical visual aura.
Sexual preference
MBA occurs in men and women of all ages but is most frequent in in adolescent girls.
Etiology
Migraine with brainstem aura is a variant of migraine with the aura symptoms arising from the brainstem or bilateral occipital hemispheres.
MBA is a complex form of migraine which itself is genetic and not well understood. Like with regular migraine, the patient’s lifestyle and environmental factors are important triggers or related issues in causation of attacks.
MBA is not usually inherited but in rare cases, due to mutation of genes, MBA my occur in more than 1 person in the family.
Genetic studies have now identified at least three genes that cause the familial form of hemiplegic migraine. These genes all cause dysfunction of ion channels on brain nerve cells and lead to an increase in the levels of glutamate, an excitatory brain neurotransmitter which relates to spreading depression.
Three genes are associated with familial hemiplegic migraine, another complicated type of migraine which comes with motor weakness on one side-“hemiplegic.” These same genes are not found in MBA.
Genetic testing is not indicated for MBA.
Kirchmann MacArthur, Lykke L, and Olesen J wrote in Neurology, 66 (6), 880-6 2006 Mar 28 “Basilar-type Migraine: Clinical, Epidemiologic, and Genetic Features.” The authors commented that “It remains uncertain whether basilar-type migraine (BM) is a subtype of migraine with typical aura (MTA) or a distinct phenotype or genotype.”
The authors found that “The patients with BM were equally distributed among the 105 families with MTA (p = 0.37). The attacks of MTA were identical in families with or without BM. No causative mutations and no linkage were identified.”
Kirchmann, et all concluded that “Basilar-type aura seemingly may occur at times in any patient with migraine with typical aura. There is no firm clinical, epidemiologic, or genetic evidence that BM is an independent disease entity different from MTA.”
Duration of attack
The aura symptoms can last from two minutes to over an hour, but not over 60 minutes.
They are then followed by a throbbing headache which is often along the back of the head, and nausea. Like regular migraine the headache part of the attack lasts from 4-72 hours.
Read my article, “What is Migraine?” on my website, www.doctormigraine.com.
Diagnosis
Diagnosis of migraine with brainstem aura (MBA) is made clinically by analysis of usual signs and symptoms. No neurologic test confirms the diagnosis, but testing should be pursued and should be normal.
Neurologic work up
Specialized blood work
24-hour heart monitoring
MRI brain scan
MRA (magnetic resonance arteriogram) scan of cerebral arteries.
Electroencephalogram (EEG)
Differential Diagnoses:
Any disorder interrupting vertebrobasilar vasculature
Vestibular Migraine
Hemiplegic Migraine
Posterior brain transient ischemic attack (TIA)
Vertebral artery dissection or thrombosis
Seizures
CADASIL
MELAS
Posterior fossa vascular and congenital abnormalities
Arteriovenous Malformation (AVM)
Arnold-Chiari malformations
Cavernous angioma
Platybasia
Comment-Migraine with brainstem aura can be confused with another rare migraine aura variant termed hemiplegic migraine. Individuals who have hemiplegic migraine experience weakness typically of the arm and face as part of their migraine aura in contrast to more common migraine aura like visual scintillations or scotoma.
It is not uncommon for people with hemiplegic migraine to have associated basilar migraine like symptomatology during their hemiplegic migraine attack in addition to weakness. The duration of hemiplegic migraine and basilar-type migraine aura symptoms is typically longer than the visual aura experienced by most.
Treatment
Triptans or drugs with ergotamine or dihydroergotamine should not be used. I know of no advice on the use of the new (2021) Gepants or Ditans.
For acute therapy simple analgesics like nonsteroidal anti-inflammatory drugs (NSAIDs) and antiemetic medications such as Phenergan (promethazine) or Zofran (ondansetron) may help with nausea.
For preventive therapy previous medical articles have suggested verapamil or topiramate. There are no randomized trials regarding how best to treat migraine with brainstem aura due to its infrequent occurrence. MBA attacks are rare, and sort of a one-time only event so prevention is usually a moot point.
There is no published data on treatment of MBA with Gepants or CGRP drugs such as Aimovig, Ajovy, or Emgality.
Prognosis
Attacks of MBA may decrease with age and since MBA is rare there is not much published on the long-term outcome for this disorder. The attacks are severe but usually resolve on their own without permanent complication.
However, like migraine with aura MBA carries a slight risk for stroke or what may be called “migrainous infarction.”
History
Chun-Pai Yang MD PhD and Shuu-Jiun Wang MD wrote on the history of Migraine with brainstem aura in Neurology Medlink, Originally released September 2, 1994; last updated September 5, 2019. The authors comments follow:
In 1961, Bickerstaff was the first to propose the concept of “basilar artery migraine” (Bickerstaff 1961a). He found 2 patients with identical symptoms that were only explicable on the basis of an abnormality of basilar artery circulation (Bickerstaff 1962).
One of these cases involved a 14-year-old whose symptoms lasted a few hours and were repeated on numerous occasions. The other involved an elderly man whose symptoms progressed rapidly to coma and death, and thrombotic occlusion of the basilar artery with infarction in brainstem and occipital cortex was demonstrated at autopsy.
So, it was by clinical analogy with the structural lesion in the basilar artery and the symptoms of basilar artery territory ischemia that the syndrome “basilar artery migraine” was first described.
Gowers probably provided the first case history of migraine with brainstem aura in the medical literature (Gowers 1907).
A female patient began to have right-sided migrainous attacks at the age of 18 years. Ten years later, these attacks changed, and she began to lose the sight in both eyes (“a black curtain seemed to be dropped down, brilliant with thousands of golden points”); she then experienced severe vertigo and dysesthesia in the arms, legs, and jaw, all lasting 10 minutes. Next, she became truly unconscious for 15 minutes and then recovered with severe headache spreading from the mastoids over the occipital region, which lasted for 2 hours.
The International Headache Society reclassified this disorder as basilar-type migraine in 2004 to replace the terminology of “basilar artery migraine,” “basilar migraine,” “Bickerstaff migraine,” and “syncopal migraine,” because involvement of the basilar artery territory is uncertain (Headache Classification Committee of the International Headache Society 2004).
The diagnostic criteria are similar to those for migraine with aura, except that the aura symptoms clearly originate from the brainstem or bilateral occipital lobes. The symptom of “bilateral paresis” was eliminated from the criteria, in order to separate this disorder from hemiplegic migraine.
In 2013, the International Headache Society reclassified this disorder as migraine with brainstem aura to replace the terminology of “basilar-type migraine” (Headache Classification Committee of the International Headache 2013).
Following ICHD-3 beta, ICHD-3 is published in 2018, and the diagnostic criteria for migraine with brainstem aura are listed above on page 1 of this article.
Bickerstaff recognized in his personal series of 300 migraine patients 34 whose “clinical symptoms suggested involvement of the basilar system in greater or lesser degree.” He realized in his patients that:
The positive ill-formed visual hallucinations in homonymous fields at times closely resemble those produced by structural lesions of the occipital lobe, and some of the hemianopic manifestations are almost certainly due to involvement of the posterior cerebral artery.
This, however, is the territory not of the internal carotid, but of the basilar artery; and there seems no reason why, if one branch of this vessel should often be involved, others--or even the main vessel itself--should not be involved as well.
Bickerstaff Case 1. A 13-year-old girl had had four menstrual periods, and three days after the end of each had had an attack in which she experienced vivid flashes of light throughout the whole visual field in both eyes. These flashes were sufficiently intense to obscure her vision completely.
At the same time, she had tingling in both hands and both feet, and her speech became so slurred as to be barely intelligible. She then became ataxic on attempting to walk. These symptoms lasted fifteen minutes and then subsided and were followed by severe throbbing occipital headache and vomiting. After vomiting, she felt better, and would sleep for several hours and awaken free from headache. Her father and an aunt had severe migraine.
In a review of “Basilar Artery Migraine” in Headache in 1985 Dr. Bickerstaff noted that a clear-cut clinical syndrome of basilar artery migraine had developed since his first report in 1961. Most patients who suffered with this type of migraine were teenagers when the first attack occurred. However, attacks could persist in migraineurs past middle age. The syndrome occurred in women and men with the usual preponderance of migraines occurring more frequently in women.
The course of an attack is similar to other aura type symptoms of migraine in that the intense neurologic symptoms last 10 to 45 minutes, then pass away, to be followed by headache with or without emesis. If the patient falls into a deep sleep, there is resolution of all symptoms upon awakening.
The visual symptoms of teichopsia, flashing lights, wavy lines, or negative scotomas such as graying of vision or visual loss usually herald the onset of the attack. An important difference from the visual involvement that occurs with migraine with aura is that with migraine with brainstem aura the whole visual field in both eyes is involved eventually even if the onset of the attack is partially hemianopic.
Short duration, complete loss of vision is not uncommon. The numbness and/or tingling affect the mouth, hands, and feet to just above the wrist and ankles. Numbness which is bilateral comes after the visual symptoms. Right and left sides of the mouth and the tongue are affected. Next vertigo, and less often tinnitus, occur and the patient develops a midline cerebellar type syndrome with gait ataxia such as would be seen with involvement of the cerebellar vermis in alcoholism.
The patient who develops dysarthria or slurring of speech with an off-balance gait does give an appearance of drunkenness. Dr. Bickerstaff noted that patients who have attacks while driving have had difficult encounters with police.
Some patients develop impairment of consciousness which may be mild drowsiness or coma such as with a stroke or head injury. Some patients appear asleep although the difference is, they may be aroused only to slip back again when stimulation stops.
Like the symptoms of migraine with aura the symptoms of migraine with brainstem aura usually regress in the order of appearance. Thus, vision returns, the numbness ceases, and then ataxia and dysarthria resolve.
A severe throbbing neck and occipital located headache ensues, sometimes extending to the whole head and accompanied by severe vomiting. Untreated, the headache may last hours and improves with sleep. Finally, the patient rallies back awake feeling exhausted.
An important point here is that no motor weakness is allowed with migraine with brainstem aura, a feature that differentiates it from hemiplegic migraine. Rarely patients with this syndrome may have permanent hemianopsias due to infarcts in the distribution of the posterior cerebral artery. Bernsen, et al, in 1990 in an article in Headache entitled “Basilar Artery Migraine Stroke” described a 25-year-old woman who had experienced migraine with brain stem aura attacks that resulted in a stroke. CAT scan of the cerebellar hemispheres revealed bilateral hypodense lesions.
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Literature Review
ICDH 3 Beta 1.2.2 Migraine with brainstem aura
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Britt Talley Daniel MD