Moyamoya disease

Case contributed by Pranav Sharma
Diagnosis certain

Presentation

Recurrent transient ischemic attacks causing left arm, leg and face weakness.

Patient Data

Age: 40 years
Gender: Female

CT angiogram

ct

Bilateral M1 occlusion with 'puff of smoke' type appearance of the proximal M1 MCA arteries bilaterally consistent with tiny collateral vessels seen in moyamoya disease. Trifurcation of A2 ACAs. Circle of Willis is otherwise patent. Left vetebral artery dominance. No aneurysm identified. 

MR angiogram

mri

Bilateral M1 MCA occlusion, suggesting moyamoya syndrome. The distal right A1 ACA also appears attenuated. The posterior communicating arteries are small. 

Cerebral angiogram

dsa

High grade right M1 segment stenosis and complete occlusion proximal left M1 segment origin. There are well formed collaterals around these abnormalities and there is well formed collateral circulation from the anterior cerebral arteries bilaterally and also posterior cerebral arteries to provide circulation to the MCA circulation. Findings would be in keeping with an angiographic diagnosis of Moyamoya. Findings are more severe on the left. 

Case Discussion

This case is of a 40-year-old female that presented to ED with recurrent TIA episodes causing left arm, leg and face weakness with dysarthria (despite angiographically the left side seems more severe).

MRA and CTA demonstrated bilateral M1 occlusion with a 'puff of smoke' appearance. The patient underwent a cerebral angiogram which confirmed high-grade M1 occlusion bilaterally with the formation of multiple small collaterals from the anterior and posterior cerebral arteries. The left side was more severe. These imaging findings were consistent with moyamoya syndrome. 

She was admitted under the Stroke team for medical management. Telemetry for 4 days did not reveal atrial fibrillation. MR brain did not show any evidence of a new stroke (DWI negative). Dual antiplatelet therapy was commenced as well as high dose statin. The patient had a negative vasculitis and thrombophilia screen. 

The patient was discussed in our hospital's Cerebrovascular Meeting and is booked for extracranial-intracranial (EC-IC) bypass surgery. 

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