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

• Published in: Neurology Vol. 64; no. 12; p. 2145
• Main Authors: Dreier, J P, Jurkat-Rott, K, Petzold, G C, Tomkins, O, Klingebiel, R, Kopp, U A, Lehmann-Horn, F, Friedman, A, Dichgans, M
• Format: Journal Article
• Language: English
• Published: United States 28.06.2005
• Subjects: Adult; Aphasia - genetics; Aphasia - pathology; Aphasia - physiopathology; Blood-Brain Barrier - pathology; Blood-Brain Barrier - physiopathology; Brain Edema - genetics; Brain Edema - pathology; Brain Edema - physiopathology; Cerebral Arteries - pathology; Cerebral Arteries - physiopathology; Cerebral Cortex - blood supply; Cerebral Cortex - pathology; Cerebral Cortex - physiopathology; Coma - genetics; Coma - pathology; Coma - physiopathology; Disease Progression; DNA Mutational Analysis; Fever - genetics; Fever - pathology; Fever - physiopathology; Functional Laterality - genetics; Hemiplegia - genetics; Hemiplegia - pathology; Hemiplegia - physiopathology; Humans; Magnetic Resonance Imaging; Male; Meninges - pathology; Meninges - physiopathology; Migraine with Aura - complications; Migraine with Aura - genetics; Migraine with Aura - physiopathology; Mutation - genetics; Sodium-Potassium-Exchanging ATPase - genetics; Time Factors
• ISSN: 1526-632X
• DOI: 10.1212/01.wnl.0000176298.63840.99

The authors report a patient with familial hemiplegic migraine type II who developed a long-lasting attack including fever, right-sided hemiplegia, aphasia, and coma. Quantitative analysis of early gadolinium-enhanced MRI revealed a mild but significant left-hemispheric blood-brain barrier (BBB) opening limited to the cortex and preceding cortical edema. The findings suggest that the delayed cortical edema was vasogenic in the severe migraine aura variant of this ATP1A2 mutation carrier.