A 3-year-old boy presented with progressively decreased mobility of the right arm. Birth and development were normal for age, and family history was negative. His symptoms started after an episode of prolonged crying, followed by vomiting. One day later, he showed irritability, dysarthria, and right hemiplegia. His parents reported a similar episode 6 months earlier that occurred in the context of febrile illness. At hospital admission, the patient was irritable. Neurological exam revealed mild right hemiparesis. Reflexes were 3+ on the right and 2+ on the left, without clonus. Plantar responses were downgoing.

Extensive coagulopathy workup showed mildly hypochromic microcytic anemia. Echocardiography was normal. Head CT was unrevealing.

Brain MRI showed subtle atrophy of the left cerebral hemisphere with overlying FLAIR-hyperintense leptomeningeal vessels (“ivy sign”). There were scattered acute left cerebral microinfarcts on a background of chronic bilateral white matter FLAIR hyperintensities ( Fig. 24.1 ).

Moyamoya disease. Brain MRI, (A and B) axial T2, (C and D) FLAIR, and (E and F) DWI, show mild atrophy of the left cerebral hemisphere ( white arrows ) with overlying FLAIR-hyperintense leptomeningeal collaterals ( white arrowheads ). There are multiple acute left cerebral microinfarcts with restricted diffusion ( black arrows ) on a background of chronic bilateral white matter changes ( black arrowheads ). DWI , Diffusion-weighted imaging; FLAIR , fluid-attenuated inversion recovery.

Magnetic resonance angiography (MRA) showed irregular stenosis of the left greater than right distal ICA, left MCA, and both anterior cerebral arteries (ACAs) ( Fig. 24.2 ).

Moyamoya disease. Coronal brain MRA shows irregular narrowing of the left greater than right distal ICAs, left MCA, and both ACAs ( white arrows ). ACAs , Anterior cerebral arteries; ICAs , internal carotid arteries; MCA , middle cerebral artery.

Head CTA and cerebral angiography showed left greater than right distal ICA stenosis with tortuous bilateral lenticulostriate moyamoya collaterals ( Fig. 24.3 ).

Moyamoya disease. (A) Coronal head CTA, (B) cerebral angiography of the right and left (C) internal carotid arteries show stenosis of both distal ICAs, A1, and M1 ( arrowheads ) with tortuous lenticulostriate moyamoya vessels bilaterally ( arrows ). ICA , Internal carotid artery.

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