We present genetically identical twin patients who experienced late-onset migraine with visual and somatosensory auras and later developed hemiplegic migraines associated with severe cortical oedema and enhancement. Both positron emission tomography and electroencephalography showed an increase in activity contralateral to the hemiplegic side. Brain biopsy during the attack showed reactive astrogliosis and microgliosis. Mutations in CACNA1A, ATP1A2, SLC1A3 and NOTCH3 were ruled out by sequencing. This report shows the clinical and genetic evaluation of a severe form of familial hemiplegic migraine as well as the evolution of the imaging changes.
Bibliographical noteFunding Information:
This project was funded by NIH/NIDCD grant P50DC05224, NIH grant 5U54RR019482 and the Clinical Research Training Fellowship grant from the American Academy of Neurology.
- Cortical oedema
- Hemiplegic migraine
- Migraine with aura