Opening of the blood-brain barrier preceding cortical edema in a severe attack of FHM type II.
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.
Dreier
J P
JP
Department of Neurology, Charité University Medicine, 10117 Berlin, Germany. jens.dreier@charite.de
Jurkat-Rott
K
K
Petzold
G C
GC
Tomkins
O
O
Klingebiel
R
R
Kopp
U A
UA
Lehmann-Horn
F
F
Friedman
A
A
Dichgans
M
M
D000328:Adult; D001037:Aphasia / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D001812:Blood-Brain Barrier / Q000473:pathology / Q000503:physiopathology*; D001929:Brain Edema / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D002536:Cerebral Arteries / Q000473:pathology / Q000503:physiopathology*; D002540:Cerebral Cortex / Q000098:blood supply / Q000473:pathology / Q000503:physiopathology*; D003128:Coma / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D004252:DNA Mutational Analysis; D018450:Disease Progression; D005334:Fever / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D007839:Functional Laterality / Q000235:genetics; D006429:Hemiplegia / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008578:Meninges / Q000473:pathology / Q000503:physiopathology; D020325:Migraine with Aura / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D009154:Mutation / Q000235:genetics; D000254:Sodium-Potassium-Exchanging ATPase / Q000235:genetics; D013997:Time Factors
C498531:ATP1A2 protein, human; D000254:Sodium-Potassium-Exchanging ATPase