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Updated: Feb 6, 2026

3D-Neuronavigation In Vivo Through a Patient's Brain During a Spontaneous Migraine Headache
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Multimodal imaging findings during severe attacks of familial hemiplegic migraine type 2

Christian Roth1, Andreas Ferbert2, Monika Huegens-Penzel3

  • 1Department of Neurology, DRK-Kliniken Nordhessen, Kassel, Germany.

Journal of the Neurological Sciences
|August 12, 2018
PubMed
Abstract

Insights

Imaging studies reveal that swelling and cortical hyperintensity in the affected hemisphere are common during Familial Hemiplegic Migraine type 2 (FHM2) attacks. These findings may correlate with prolonged FHM2 attack duration.

Area of Science:

  • Neurology
  • Neuroimaging
  • Genetics

Background:

  • Familial hemiplegic migraine (FHM) is a rare genetic migraine with aura.
  • Three subtypes exist: FHM1, FHM2, and FHM3.
  • Limited imaging data is available for FHM2 during acute attacks.

Purpose of the Study:

  • To investigate neuroimaging findings in patients with genetically confirmed FHM2 during acute attacks.
  • To compare imaging findings in FHM2 with those previously reported in FHM1.
  • To explore potential imaging correlates of prolonged FHM2 attack duration.

Main Methods:

  • Retrospective analysis of imaging data from four FHM2 patients.
  • Inclusion of eight cMRI and two CT perfusion studies from six different attacks.
  • Evaluation of cMRI for cytotoxic edema and T2/FLAIR hyperintensity; CT perfusion for hemisphere perfusion.

Main Results:

  • No evidence of cytotoxic edema on cMRI.
  • Prominent finding: swelling and/or cortical hyperintensity in the affected hemisphere on T2/FLAIR MRI (observed in 4 attacks across 3 patients).
  • Additional findings: increased perfusion and middle cerebral artery dilatation in the affected hemisphere in some patients.

Conclusions:

  • Cortical hyperintensity and swelling in the affected hemisphere, previously noted mainly in FHM1, are also observed in FHM2.
  • These imaging changes appear later in attacks, not at the onset.
  • The observed imaging findings may be associated with the prolonged attack duration seen in FHM2 patients.
Keywords:
ATP1A2CTFHM2Familial hemiplegic migraineMRIPerfusion

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