Focal seizures and recurrent episodes of ischaemia.
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MR demonstrates bilateral frontal cortical border zone infarcts with encephalomalacia and gliosis. No diffusion restriction was noted.
3D TOF MRA shows severe stenosis involving the supraclinoid segments of the internal carotid arteries. Anterior cerebral arteries (ACA) and middle cerebral arteries (MCA) not visualized along with its cortical branches. There are multiple small thalamostriate perforators collaterals giving the typical puff of smoke appearance.
This case demonstrates typical findings of Moyamoya disease with classical "puff" or "cloud of smoke" sign.