title: Biphasic neurovascular changes in prolonged migraine aura in familial hemiplegic migraine type 2.
abstract: OBJECTIVE
  To report biphasic changes in cerebral blood flow (CBF) in the acute phase of hemiplegic migraine with prolonged aura (HMPA), in which aura symptoms lasted longer than 24 h, in three patients with familial hemiplegic migraine (FHM) carrying a p.H916L mutation in ATP1A2 gene.
  
  
  METHODS
  We assessed neurovascular changes with time in the affected cerebral hemisphere corresponding to aura symptoms during the acute phase of HMPA. Arterial spin labelling MRI, SPECT for CBF measurement and EEG in three attacks, in one attack FDG-PET measurement for cerebral metabolism was performed. We evaluated CBF at different phases of aura symptoms in 11 attacks of HMPA.
  
  
  RESULTS
  In two attacks, we found biphasic CBF changes beginning with hypoperfusion followed by persistent hyperperfusion. FDG-PET revealed increased cerebral glucose metabolism in the regions corresponding to hyperperfusion on day 4 when aura symptoms still persisted. In four attacks, Z-score-based CBF mapping revealed multifocal hypoperfusion in the early phase. Hypoperfusion in our study was seen within 19 h of the onset of the symptoms in five of seven attacks, while hyperperfusion was seen 18 h or later in eight of nine attacks. EEG showed attenuated alpha activity without paroxysmal discharge.
  
  
  CONCLUSIONS
  This is the first report showing biphasic CBF changes during the prolonged aura of FHM2. This study suggested that the results of cross-sectional CBF studies should be interpreted carefully. Initial multifocal hypoperfusion is likely due to functional depression of multifocal origin in the affected hemisphere, but the mechanism of persistent hyperperfusion requires further investigation.
authors.affiliation: Department of Neurology, Kitasato University School of Medicine, Sagamihara, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Iizuka
authors.affiliation: Department of Neurology, Kitasato University School of Medicine, Sagamihara, Japan.
authors.forename: Naomi
authors.initials: N
authors.lastname: Tominaga
authors.affiliation: Department of Neurology, Kitasato University School of Medicine, Sagamihara, Japan.
authors.forename: Juntaro
authors.initials: J
authors.lastname: Kaneko
authors.affiliation: Department of Neurology, Kitasato University School of Medicine, Sagamihara, Japan.
authors.forename: Mayumi
authors.initials: M
authors.lastname: Sato
authors.affiliation: Department of Neurology, Kitasato University School of Medicine, Sagamihara, Japan.
authors.forename: Tsugio
authors.initials: T
authors.lastname: Akutsu
authors.affiliation: Department of Neurology, Kitasato University School of Medicine, Sagamihara, Japan.
authors.forename: Junichi
authors.initials: J
authors.lastname: Hamada
authors.affiliation: Saitama International Headache Center, Saitama Neuropsychiatric Institute, Saitama, Japan.
authors.forename: Fumihiko
authors.initials: F
authors.lastname: Sakai
authors.affiliation: Department of Neurology, Kitasato University School of Medicine, Sagamihara, Japan.
authors.forename: Kazutoshi
authors.initials: K
authors.lastname: Nishiyama
mesh_terms: D000328:Adult; D000368:Aged; D000513:Alpha Rhythm; D001921:Brain / Q000098:blood supply*; D002545:Brain Ischemia / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D004252:DNA Mutational Analysis; D004292:Dominance, Cerebral / Q000502:physiology; D004569:Electroencephalography; D005260:Female; D006580:Genetic Carrier Screening; D006801:Humans; D006940:Hyperemia / Q000175:diagnosis / Q000503:physiopathology; D007090:Image Interpretation, Computer-Assisted; D021621:Imaging, Three-Dimensional; D018810:Magnetic Resonance Angiography; D008875:Middle Aged; D020325:Migraine with Aura / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology*; D009460:Neurologic Examination; D010375:Pedigree; D049268:Positron-Emission Tomography; D012039:Regional Blood Flow / Q000502:physiology; D000254:Sodium-Potassium-Exchanging ATPase / Q000235:genetics; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: C498531:ATP1A2 protein, human; D000254:Sodium-Potassium-Exchanging ATPase
keywords: Migraine; Mri; Pet; Spect