A case of hemiplegic migraine with focal cerebral atrophy in the chronic phase

<p>  In cases of familial hemiplegic migraine or epilepsy, cerebral atrophy may occur at the site of focal symptoms. We report a case of sporadic hemiplegic migraine with marked cerebral atrophy 5 years after the initial diagnosis. The patient was a man in his 50s. The patient had a history of migraine with aura. He was hospitalized three times in two years for migraines with focal symptoms such as aphasia, dyslexia, and visual field disturbances. On his first episode, he was diagnosed as transient ischemic attack and started taking Cilostazol, which seemed inappropriate retrospectively. On his second episode, he showed general convulsion and started taking an antiseizure medication. On his third episode, he was diagnosed with hemiplegic migraine with left hemiplegia and started taking other antiseizure medications with known preventive effects on migraine. Cerebral perfusion study showed hypoperfusion at the focus of cortical spreading depression during the first episode, whereas hyper-perfusion during the second and the third episode. Brain imaging 5 years after the first visit revealed marked brain atrophy on his right occipital lobe. He passed away when he drowned due to epileptic seizure while bathing. As the mechanism of the focal brain atrophy, neuronal damage due to (1) drastic changes in cerebral perfusion that appears during aura, (2) repetitive cortical spreading depression, and (3) coexistence of epilepsy, were suggested. The neuronal damage which we observed as FLAIR high lesion at the focus of cortical spreading depression is also described as cortical cytotoxic edema or cortical laminar necrosis. Regarding the possibly poor outcome of those hemiplegic migraine patients, recently approved promising agents such as anti-calcitonin-gene related peptide (CGRP) -related monoclonal antibodies or lasmiditan should have been considered.</p>