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Moyamoya disease

Case contributed by Andrew Dixon
Diagnosis certain

Presentation

One week history of left-sided weakness.

Patient Data

Age: 3 years
Gender: Female

MRI brain

mri

The supraclinoid ICA and P1 segments of the PCA are occluded with large perforator collaterals. A number of basal ganglia infarcts are noted on DWI. 

dsa

Angiogram confirms extensive perforator colaterals giving the 'puff of smoke' characteristic of this disease, and involving both anterior and posterior circulation. 

Case Discussion

Typical features of moya moya disease in the typical age group, and presenting in the typical fashion for this age group - namely infarcts. 

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