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Opening of the blood-brain barrier preceding cortical edema in a severe attack of FHM type II

J P Dreier1, K Jurkat-Rott, G C Petzold

  • 1Department of Neurology, Charité University Medicine, 10117 Berlin, Germany. jens.dreier@charite.de

Neurology
|June 30, 2005
PubMed

Insights

Familial hemiplegic migraine type II, a severe aura variant, can cause prolonged attacks with neurological deficits. This case highlights blood-brain barrier opening and vasogenic edema in the cortex, linked to ATP1A2 mutations.

Area of Science:

  • Neurology
  • Neuroimaging
  • Genetics

Background:

  • Familial hemiplegic migraine (FHM) is a rare subtype of migraine with aura, often associated with genetic mutations.
  • FHM type II is specifically linked to mutations in the ATP1A2 gene, which encodes a sodium-potassium ATPase alpha-2 subunit.

Observation:

  • A patient with FHM type II experienced a severe, prolonged migraine attack characterized by fever, right-sided hemiplegia, aphasia, and coma.
  • Early gadolinium-enhanced MRI revealed a subtle but significant opening of the blood-brain barrier (BBB) in the left cerebral cortex.
  • This BBB opening was followed by the development of cortical edema.

Findings:

  • Quantitative MRI analysis demonstrated a localized BBB disruption preceding cortical edema.
  • The findings suggest a vasogenic mechanism for the delayed cortical edema in this severe migraine aura variant.
  • The observed BBB opening is directly related to the ATP1A2 mutation in this patient.

Implications:

  • This case provides novel insights into the pathophysiology of severe FHM aura variants.
  • Understanding BBB dynamics in FHM can inform diagnostic approaches and potential therapeutic strategies.
  • Further research into ATP1A2 mutations and their effect on BBB integrity is warranted for FHM patients.

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