Migraine with Brainstem Aura

Bickerstaff,[i] in his seminal article entitled, “Basilar Artery Migraine” published in The Lancet in 1961 began with the following statement from Aretaeus (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.

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.

Case 1 of his 4 clinical cases is reproduced below:

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 [ii] 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,[iii] 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.

Treatment of migraine with brainstem aura is the same as treatment of other forms of migraine, except that triptans are contraindicated by the FDA.  PDR and Product Information originally limited triptans for basilar artery migraine which has now been replaced with migraine with brainstem aura.  Published articles show that triptans and ergotamine work for migraine with brainstem aura and are safe.[iv]  Migraine experts do use triptans and ergotamine for treatment considering that the symptoms of migraine with brainstem aura are rare types of migraine, but are still migraine and like migraine with aura the symptoms are not related to arterial vasoconstriction, but rather to spreading depression. Hemiplegic migraine is accompanied by a paralysis of one side of the body. Symptoms of brainstem dysfunction (double vision, unsteady gait, vertigo, difficulty speaking) made doctors think that ischemia or lack of blood flow in that artery caused these symptoms. We now know that this is not the case and that migraine with brainstem aura is just another form of migraine with aura.

Some doctors are afraid to prescribe triptans to such patients out of fear of litigation.  ICDH 3 beta no longer includes basilar migraine because it is recognized as being just a form of migraine with aura. Ergots and triptans are not contraindicated in migraine with and without aura.  Also, the migraine lifestyle and preventive drugs should be administered. 

ICDH 3 Beta 1.2.2 Migraine with brainstem aura

Previously used terms:
Basilar artery migraine; basilar migraine; basilar-type migraine.

Description:
Migraine with aura symptoms clearly originating from the brainstem, but no motor weakness.

Diagnostic criteria:
A. At least two attacks fulfilling criteria B-D
B. Aura consisting of visual, sensory and/or speech/language symptoms, each fully reversible, but no motor1 or retinal symptoms
C. At least two of the following brainstem symptoms:

1. dysarthria
2. vertigo
3. tinnitus
4. hypacusis
5. diplopia
6. ataxia
7. decreased level of consciousness

D. At least two of the following four characteristics:

1. at least one aura symptom spreads gradually over ≥5 min, and/or two or more symptoms occur in succession
2. each individual aura symptom lasts 5-60 min2
3. at least one aura symptom is unilateral3
4. the aura is accompanied, or followed within 60 min, by headache

E. Not better accounted for by another ICHD-3 diagnosis, and transient ischaemic attack has been excluded.

Notes:
1. When motor symptoms are present, code as 1.2.3 Hemiplegic migraine.

2. When for example three symptoms occur during an aura, the acceptable maximal duration is 3×60 minutes.

3. Aphasia is always regarded as a unilateral symptom; dysarthria may or may not be.

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 under criterion C may occur with anxiety and hyperventilation, and therefore are subject to misinterpretation.

Literature Review

[i] Bickerstaff ER.  Basilar Artery Migraine.  Lancet.  1961;1:15-17.

[ii]  Bickerstaff ER. Basilar Artery Migraine. Headache. 1985;4(4):3-11.

[iii] Bernsen HJJA, Vlasakker CV, Verhagen WIM, Prick MJJ.  Basilar Artery Migraine Stroke.  Headache: The Journal of Head and Face Pain.  0(3)142-144.

[iv] Klapper J, Mathew N, Nett R.  Triptans in the Treatment of Basilar Migraine and Migraine With Prolonged Aura.  Headache: The Journal of Head and Face Pain. 2001;41(10): 981–984.