
Patient Background:
A 23-year-old woman with childhood-onset Moyamoya disease presented with recurrent seizures and a history of cerebrovascular events. At age 5, she experienced focal motor seizures, initially misattributed to psychogenic causes. By age 7, she developed acute right-sided weakness and aphasia, resulting in residual facial palsy and partial aphasia.
By 2023, exam findings included asthenic habitus, venous insufficiency, mild cognitive impairment (MMSE 26), hyperreflexia (right > left), distal right arm weakness, prosopagnosia, and severe myopia.
Assessment & Diagnosis:
An MRI from 2005 revealed right hemisphere atrophy. In 2006, cerebral angiography confirmed Moyamoya disease, with bilateral carotid artery occlusions and collateral “puff of smoke” vessels. Repeat MRI in 2023 demonstrated chronic ischemic lesions, and angiography confirmed diminished anterior circulation with compensatory collaterals. EEG revealed right-sided background slowing and left temporoparietal epileptiform discharges, consistent with focal seizures. Neuropsychological testing showed mild cognitive disorder with visual–spatial deficits and agnosia.
- How is seizure control balanced with stroke prevention in Moyamoya disease?
- When is revascularization considered in Moyamoya with seizures, and how is perioperative seizure risk managed?

