title: Habituation of evoked responses is greater in patients with familial hemiplegic migraine than in controls: a contrast with the common forms of migraine.
abstract: BACKGROUND
  Familial hemiplegic migraine (FHM) is a rare, dominantly inherited subtype of migraine with transient hemiplegia during the aura phase. Mutations in at least three different genes can produce the FHM phenotype. The mutated FHM genes code for ion transport proteins that animal and cellular studies have associated with disturbed ion homeostasis, altered cellular excitability, neurotransmitter release, and decreased threshold for cortical spreading depression. The common forms of migraine are characterized interictally by a habituation deficit of cortical and subcortical evoked responses that has been attributed to neuronal dysexcitability. FHM and the common forms of migraine are thought to belong to a spectrum of migraine phenotypes with similar pathophysiology, and we therefore examined whether an abnormal habituation pattern would also be found in FHM patients.
  
  
  METHODS
  In a group of genotyped FHM patients (five FHM-1, four FHM-2), we measured habituation of visual evoked potentials (VEP), auditory evoked potentials including intensity dependence (IDAP), the nociception-specific blink reflex (nsBR) and compared the results to a group of healthy volunteers (HV).
  
  
  RESULTS
  FHM patients had a more pronounced habituation during VEP (P=0.025) and nsBR recordings (P=0.023) than HV. There was no difference for IDAP, but the slope tended to be steeper in FHM.
  
  
  CONCLUSIONS
  Contrary to the common forms of migraine, FHM patients are not characterized by a deficient, but rather by an increased habituation in cortical/brain stem evoked activities. These results suggest differences between FHM and the common forms of migraine, as far as central neuronal processing is concerned.
authors: Danish Headache Center, Department of Neurology, Glostrup Hospital, Faculty of Health Sciences, University of Copenhagen, DK-2600 Glostrup, Copenhagen, Denmark.
authors: J M
authors: JM
authors: Hansen
authors: M
authors: M
authors: Bolla
authors: D
authors: D
authors: Magis
authors: V
authors: V
authors: de Pasqua
authors: M
authors: M
authors: Ashina
authors: L L
authors: LL
authors: Thomsen
authors: J
authors: J
authors: Olesen
authors: J
authors: J
authors: Schoenen
mesh_terms: D000328:Adult; D005071:Evoked Potentials / Q000502:physiology*; D006185:Habituation, Psychophysiologic / Q000502:physiology*; D006801:Humans; D008875:Middle Aged; D008881:Migraine Disorders / Q000503:physiopathology*; D020325:Migraine with Aura / Q000235:genetics / Q000503:physiopathology*; D012815:Signal Processing, Computer-Assisted; D055815:Young Adult