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Moyamoya disease

Case contributed by Dr Mostafa El-Feky



Patient Data

Age: 35 years
Gender: Female


Intraventricular and intraparenchymal hemorrhage noted at the right temporoparietal region, extending to the body of right lateral ventricle. It displays T1 and T2 hyperintense signal with a hypointense rim. It shows blooming in SWI and DWI restriction.

MRA revealed occlusion of the pre-communicating segment of both carotid arteries; also A1 and M1 segments of both ACAs and MCAs with extensive lenticulostriate collaterals recanalizing its distal segments forming puff of smoke appearance

Scattered white matter T2 hyperintensities are noted mainly involving the periventricular area and centrum semiovale, in keeping of deep white matter ischemic changes.

Case Discussion

This patient had a known diagnosis of Moyamoya disease, an idiopathic vascular occlusive disease, which causes progressive stenosis of the terminal internal carotid artery bifurcation including the proximal anterior and middle cerebral arteries in association with the development of dilated perforating arteries that function as collateral pathways. 

In adults, hemorrhage from the abnormal vessels can occur resulting in intracerebral or intraventricular hematoma as seen in this case. The common site of hemorrhage is intraventricular and in the paraventricular area of the posterior lateral ventricle which is the blood supply area of the lateral posterior choroidal artery 1.

It is worth learning to recognize it on non-vascular imaging, and T2 is best for this. 

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Case information

rID: 73035
Published: 29th Dec 2019
Last edited: 13th Jun 2021
Inclusion in quiz mode: Included
Institution: Dar El Ashaa Center

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