Migraine with brainstem aura: defining the core syndrome

Area of Science:

• Neurology
• Headache Medicine

Background:

• Migraine with brainstem aura is a rare subtype of migraine with aura, with diagnostic challenges due to symptom overlap.
• Existing diagnostic criteria are considered too permissive, potentially including cortical symptoms.

Purpose of the Study:

• To determine if true brainstem aura exists and its prevalence.
• To evaluate the adequacy of current diagnostic criteria for migraine with brainstem aura.
• To develop refined criteria for a core syndrome of brainstem aura.

Main Methods:

• Systematic review of literature cases.
• Analysis of clinical cases from the Danish Headache Center (DHC) including interviews, examinations, and imaging.
• Evaluation of a large cohort diagnosed via telephone interview.

Main Results:

• A review of 79 detailed literature cases found 44 met ICHD-3 criteria.
• At the DHC, 1.37% of migraine with aura cases (0.04% of the general population) met criteria.
• Telephone interviews yielded a higher diagnosis rate, indicating overdiagnosis with current criteria.
• Stricter criteria were developed to define a core syndrome of brainstem aura.

Conclusions:

• Migraine with brainstem aura is a rare but distinct neurological entity.
• Current diagnostic criteria are not specific enough for this rare migraine subtype.
• New, stricter criteria can better define the core syndrome of migraine with brainstem aura, likely linked to brainstem dysfunction.