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Updated: May 13, 2026

A Thrombotic Stroke Model Based On Transient Cerebral Hypoxia-ischemia
06:01

A Thrombotic Stroke Model Based On Transient Cerebral Hypoxia-ischemia

Published on: August 18, 2015

Dissociated neurovascular dynamics in ATP1A2-related familial hemiplegic migraine mimicking acute ischemic stroke

Gha-Hyun Lee1,2, Jiyoung Kim3,4, Jae Wook Cho4,5

  • 1Department of Neurology, Biomedical Research Institute, Pusan National University Hospital, 179 Gudeok-ro, Seo-gu, Busan, 49241, Korea, Republic Of. ka-hyun-ii@hanmail.net.

BMC Neurology
|May 12, 2026
PubMed
Abstract

Insights

Familial hemiplegic migraine type 2 (FHM2) can mimic stroke. This case shows a unique neurovascular dissociation with reduced large vessel flow and increased small vessel flow, aiding diagnosis and preventing incorrect stroke treatment.

Area of Science:

  • Neurology
  • Neuroimaging
  • Genetics

Background:

  • Hemiplegic migraine (HM) is a rare migraine with motor aura.
  • Familial hemiplegic migraine type 2 (FHM2), linked to ATP1A2 mutations, can resemble acute stroke.
  • Differentiating FHM2 from stroke using neuroimaging is crucial due to distinct underlying pathophysiology.

Purpose of the Study:

  • To characterize the neurovascular dynamics in a patient with FHM2.
  • To highlight the unique dissociated perfusion pattern observed in FHM2.
  • To emphasize the importance of recognizing this pattern for differential diagnosis.

Main Methods:

  • Case presentation of a 37-year-old man with acute headache and aphasia.
  • Utilized diffusion-weighted MRI, time-of-flight MRA, and SWI to assess vascular flow and venous congestion.
  • Performed EEG and genetic testing to confirm ATP1A2 mutation consistent with FHM2.

Main Results:

  • MRI revealed attenuated large artery signal (TOF-MRA) and prominent cortical veins (SWI) in the left hemisphere.
  • EEG showed left hemispheric slowing.
  • Genetic testing confirmed a pathogenic ATP1A2 mutation, diagnosing FHM2.

Conclusions:

  • FHM2 presents with a unique neurovascular dissociation: reduced macrovascular flow and presumed microvascular hyperemia.
  • This pattern results from ATP1A2-related astrocytic dysfunction and cortical spreading depression.
  • Identifying this reversible pattern is vital to differentiate FHM2 from stroke mimics and avoid unnecessary thrombolysis.
Keywords:
ATP1A2 mutationCortical spreading depressionFamilial hemiplegic migraineNeurovascular couplingSusceptibility-weighted imaging

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