Pediatric Moya Moya Disease

  • Etiology: idiopathic
  • Imaging: occlusion of internal carotid terminus, reconstitution of M1/A1 via thalmoperforators (puff of smoke) and cortical collaterals
  • MRI: Failure to suppress sulcal CSF on FLAIR, sulcal enhancement, dilated perforators in diencephalon, T2 hypointense white matter under ischemic cortex
  • Clinical: presentation in childhood – seizure / TIA / ischemic stroke, presentation in adults – hemorrhagic stroke

Radiology Cases of Moya Moya Disease

MRA of Moya Moya disease
AP view of a 3D MR angiogram with contrast of the brain shows marked narrowing of the bilateral supraclinoid internal carotid arteries and nonvisualization of the A1 and M1 segments of the anterior and middle cerebral arteries with multiple lenticulostriate collateral vessels arising from the terminal portions of the supraclinoid internal carotid arteries which are reconstituting the A2 and M2 segments.