Serial magnetic resonance imaging findings during severe attacks of familial hemiplegic migraine type 2: a case report

BMC Neurol. 2021 Apr 21;21(1):173. doi: 10.1186/s12883-021-02201-z.


Background: Hemiplegic migraines represent a heterogeneous disorder with various presentations. Hemiplegic migraines are classified as sporadic or familial based on the presence of family history, but both subtypes have an underlying genetic etiology. Mutations in the ATP1A2 gene are responsible for Familial Hemiplegic type 2 (FHM2) or the sporadic hemiplegic migraine (SHM) counterpart if there is no family history of the disorder. Manifestations include migraine with aura and hemiparesis along with a variety of other symptoms likely dependent upon the specific mutation(s) present.

Case presentation: We report the case of an adult man who presented with headache, aphasia, and right-sided weakness. Workup for stroke and various infectious agents was unremarkable during the patient's extended hospital stay. We emphasize the changes in the Magnetic Resonance Imaging (MRI) over time and the delay from onset of symptoms to MRI changes in Isotropic Diffusion Map (commonly referred to as Diffusion Weighted Imaging (DWI)) as well as Apparent Diffusion Coefficient (ADC).

Conclusions: We provide a brief review of imaging findings correlated with signs/symptoms and specific mutations in the ATP1A2 gene reported in the literature. Description of the various mutations and consequential presentations may assist neurologists in identifying cases of Hemiplegic Migraine, which may include transient changes in ADC and DWI imaging throughout the course of an attack.

Keywords: ATP1A2; FHM; Hemiplegic; MRI; Migraine.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brain / diagnostic imaging
  • Brain / pathology
  • Diffusion Magnetic Resonance Imaging / methods
  • Humans
  • Male
  • Migraine with Aura / diagnostic imaging*
  • Migraine with Aura / genetics
  • Migraine with Aura / pathology*
  • Mutation
  • Neuroimaging / methods
  • Sodium-Potassium-Exchanging ATPase / genetics


  • ATP1A2 protein, human
  • Sodium-Potassium-Exchanging ATPase

Supplementary concepts

  • Hemiplegic migraine, familial type 2