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  • Updated 02.15.2022
  • Released 09.02.1994
  • Expires For CME 02.15.2025

Migraine with brainstem aura

Introduction

Overview

In response to advances in research and treatment, an evolving International Classification of Headache Disorders (ICHD) system is available for physicians to properly diagnose headache disorders. What was formerly known as “basilar-type migraine,” “basilar migraine,” “basilar artery migraine,” or “Bickerstaff migraine” is now referred to as migraine with brainstem aura. Because the involvement of the basilar artery is unlikely, the term migraine with brainstem aura is preferred. The associated symptoms clearly originate from the brainstem and are not ischemic in etiology. According to the ICHD-3, the diagnosis is based on the findings of full reversible speech or language, sensory, or visual auras that are not accompanied by retinal or motors symptoms. There must be at least two of the following “brainstem” symptoms present: dysarthria, vertigo, tinnitus, hypacusis, diplopia, ataxia not attributable to sensory deficit, and decreased level consciousness (Glasgow Coma Scale ≤13). By definition, the aura symptoms spread over 5 or more minutes, last from 5 to 60 minutes, and may be followed by a headache within 1 hour. Some authors have proposed requiring three rather than the current criteria of two brainstem aura symptoms for diagnosis, while setting a more stringent criterion of combining hearing loss and tinnitus into a single auditory feature, criterion B ("hearing loss and/or tinnitus") (75; 46). Otherwise, a patient with vestibular migraine and vertigo attacks could easily meet the diagnosis of migraine with brainstem aura when the patient also has hearing loss and tinnitus onset. Although migraine with brainstem aura is a rare subtype of migraine, it is the most common variant of migraine with aura and occurs in 1.5% of patients with headache. The onset of the disease usually occurs at the second or third decade. The differential diagnosis for this disorder includes any condition that can cause symptoms and signs referable to posterior circulation insufficiency, including transient ischemic attack, vertebral dissection, or thrombosis. Furthermore, seizures, CADASIL, MELAS, and the posterior fossa vascular and congenital abnormalities, including arteriovenous malformations, Chiari malformations, cavernous angioma, or platybasia may need to be excluded by MRI. 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 two migraine attacks accompanied by at least two of the following fully reversible symptoms: dysarthria, vertigo, tinnitus, hypacusis, diplopia, ataxia not attributable to sensory deficit, and decreased level consciousness (Glasgow Coma Scale ≤13).

• 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 prophylactic treatments.

Historical note and terminology

In 1961, Bickerstaff was the first to propose the concept of “basilar artery migraine” (08). He found two patients with identical symptoms that were only explicable on the basis of an abnormality of basilar artery circulation (10). 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... (11).

Gowers probably provided the first case history of migraine with brainstem aura in the medical literature (30). A female patient began to have right-sided migrainous attacks at the age of 18 years. 10 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” (33). Following ICHD-3 beta, ICHD-3 is published in 2018, and the diagnostic criteria for migraine with brainstem aura are as follows (Table 1).

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

(1) Attacks fulfilling criteria for 1.2. migraine with aura and criterion B below
(2) Aura with both of the following:
(3) At least two of the following fully reversible brainstem symptoms:

(A) dysarthria
(B) vertigo
(C) tinnitus
(D) hypacusis
(E) diplopia
(F) ataxia not attributable to sensory deficit
(G) decreased level of consciousness (GCS ≤13)

(4) No motor or retinal symptoms


Notes

1. Dysarthria should be distinguished from aphasia.
2. Vertigo does not embrace and should be distinguished from dizziness.
3. This criterion is not fulfilled by sensations of ear fullness.
4. Diplopia does not embrace (or exclude) blurred vision.
5. The Glasgow Coma Scale (GCS) score may have been assessed during admission; alternatively, deficits clearly described by the patient allow GCS estimation.
6. When motor symptoms are present, code as 1.2.3 hemiplegic migraine.

(34)

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