Moyamoya disease with EDAMS

Large areas of gliosis are seen in bilateral cerebral hemispheres, predominantly in bilateral temporo-occipital lobes - representing extensive ischemic changes.  There is ex-vacuo dilatation of bilateral lateral and 3^rd ventricles.

Post operative changes are seen in the form of bilateral temporalis muscle flaps along with bilateral superficial temporal artery branches on bilateral temporal lobes - consistent with encephaloduroarteriomyosynagniosis. There is collateralization between few bilateral middle cerebral artery branches and bilateral superficial temporal artery branches.

Thin bilateral fronto-parietal extradural collections are noted.

Intracranial bilateral internal carotid arteries are diffusely small in caliber measuring 1.8mm on right and 2.0mm on left. There is severe abrupt narrowing of bilateral supraclinoid internal carotid arteries.

There is non visualization of bilateral middle cerebral arteries.  

A1 segment of left anterior cerebral artery is not visualized. Rest of the left anterior cerebral artery is small in caliber.   

Right anterior cerebral artery is not visualized.

Multiple small leptomeningeal collaterals are seen in basal cisterns.

Bilateral intracranial vertebral arteries are normal in course and caliber.

Basilar artery and both posterior cerebral arteries appear normal.  

Annotated coronal MIP image of the MR angiogram shows normal appearence of posterior circulation, hypoplastic bilateral internal carotid arteries and middle cerebral arteries. Bilateral superficial temporal artery branches are noted adjacent to bilateral temporal poles.