Moyamoya disease

Case contributed by Dr Ayaz Hidayatov

Presentation

Focal seizures and recurrent episodes of ischaemia.

Patient Data

Age: 15
Gender: Male
MRI

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.

Case Discussion

This case demonstrates typical findings of Moyamoya disease with classical  "puff" or "cloud of smoke" sign.

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Case information

rID: 59130
Published: 22nd Mar 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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