Moyamoya disease

Case contributed by Keshaw Kumar
Diagnosis certain

Presentation

Right upper limb and left lower limb weakness

Patient Data

Age: 2 years
Gender: Male

MRI Brain

mri

Moderate size ill-defined area of diffusion restriction on DWI in the right high frontal lobe in para sagittal location with corresponding hypo intensity on ADC map. A subtle patchy area of diffusion restriction is also seen in the right basal ganglia region with corresponding hypo intensity on ADC mapping, predominantly involving the caudate nucleus and putamen.----Suggestive of acute ischemic infarcts in right ACA & MCA territory.

Small patchy area of gliosis / chronic infarct in the right frontal lobe in the anterior aspect.

Moderate size ill-defined area of slightly hypointense signal alteration with cortical hyperintensity on FLAIR images in the right frontoparietal region, likely due to pial collaterals.

The distal intracranial ICA, MCA & ACA flow voids are not visualized and are replaced by multiple tiny collateral vessels in the basal cistern and peri mesencephalic cistern regions.

Few discrete and confluent FLAIR/T2W hyperintense foci in periventricular and deep white matter in bilateral fronto-parietal regions, likely chronic ischemic foci.

Minimal diffuse cerebral atrophy.

CT brain and neck angiography

ct

The distal bilateral internal carotid arteries and anterior & middle cerebral arteries are replaced by extensive collateral blood vessels in this region.

Case Discussion

Overall imaging findings are suggestive of moyamoya disease.

Co-author: Dr. Vivek Ranjan (DNB, Pediatrics).

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