Migraine with brainstem aura

Kai-Chen Wang MD (Dr. Wang of Cheng Hsin General Hospital in Taiwan has no relevant financial relationships to disclose.)
Shuu-Jiun Wang MD, editor. (Dr. Wang of the National Yang-Ming University School of Medicine and the Neurological Institute, Taipei Veterans General Hospital received consulting fees from Eli Lilly.)
Originally released September 2, 1994; last updated August 3, 2015; expires August 3, 2018

This article includes discussion of migraine with brainstem aura, basilar artery migraine, basilar migraine, basilar-type migraine, and Bickerstaff migraine. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Migraine with brainstem aura, formerly known as “basilar-type migraine,” is a variant of migraine with the aura symptoms arising from the brainstem or bilateral occipital hemispheres. The onset of the disease usually occurs at the second or third decade. The diagnosis is based on the finding of at least 2 migraine attacks accompanied by at least 2 of the following fully reversible symptoms: dysarthria, vertigo, tinnitus, impaired hearing, double vision, ataxia, and decreased level consciousness. The differential diagnosis should include cerebrovascular diseases, seizures, CADASIL, MELAS, and the pathology of posterior fossa. Despite the lack of data suggesting migraine with brainstem aura as a vasospastic condition, the use of triptans has been considered prohibited. Anticonvulsants and calcium channel blockers may be the drugs of choice in the prophylaxis.

Key points

 

• Migraine with brainstem aura is an episodic disorder and occurs in 1.5% of patients with headache.

 

• Migraine with brainstem aura is a variant of migraine with the aura symptoms arising from the brainstem or bilateral occipital hemispheres.

 

• “Basilar-type migraine” has been renamed “migraine with brainstem aura” according to ICHD-3 beta.

 

• The onset of the disease usually occurs at the second or third decade.

 

• The diagnosis is based on the finding of at least 2 migraine attacks accompanied by at least 2 of the following fully reversible symptoms: dysarthria, vertigo, tinnitus, impaired hearing, double vision, ataxia, and decreased level consciousness.

 

• The aura symptoms clearly originate from the brainstem without motor weakness.

 

• The differential diagnosis should include cerebrovascular diseases, seizures, CADASIL, MELAS, and the pathology of posterior fossa.

 

• Despite the lack of data suggesting migraine with brainstem aura as a vasospastic condition, the use of triptans has been considered prohibited. Anticonvulsants and calcium channel blockers may be the drugs of choice in the prophylaxis.

Historical note and terminology

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.

Bickerstaff attributed the earliest recorded description of basilar artery migraine to Aretaeus, who gave the following description in the first century AD:

 

If darkness possess the eye, 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 1986).

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) (Table 1).

Table 1. Diagnostic Criteria of Migraine with Brainstem Aura (ICHD-3 beta)

A. At least 2 attacks fulfilling criteria B through D

B. Aura consisting of visual, sensory and/or speech/language symptoms, each fully reversible, but no motor or retinal symptoms

C. At least 2 of the following brainstem symptoms:

 

1. Dysarthria
2. Vertigo
3. Tinnitus
4. Hypacusia
5. Diplopia
6. Ataxia
7. Decreased level of consciousness

D. At least 2 of the following 4 characteristics:

 

1. At least 1 aura symptom spreads gradually over 5 or more minutes and/or 2 or more symptoms occur in succession
2. Each individual aura symptom lasts 5 to 60 minutes
3. At least 1 aura symptom is unilateral
4. The aura is accompanied, or followed within 60 minutes, by headache

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

(Headache Classification Committee of the International Headache 2013)

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