Moyamoya disease

Case contributed by Dr Mohammad A. ElBeialy

Presentation

Girl with developmental delay presented with right-sided hemiplegia.

Patient Data

Age: 5 years
Gender: Female
MRI

Left fronto-temporo-parieto-occipital patchy cortical and subcortical areas of restricted diffusion with high DWI and low ADC signals. They show low T1 and high T2/FLAIR signal intensity. 

Periventricular as well as deep white matter patchy areas and foci of T2 and FLAIR hyperintensity are seen.

Evidence of high fronto-parietal as well as occipital leptomeningeal FLAIR hyperintensity is noted “ivy sign”. Multiple signal void collateral vessels are seen in the basal ganglia and thalamic regions.      

Mild bilateral otomastoiditis with T2 prolongation as well as enlarged nasopharyngeal adenoid.

MR angiography of the brain, revealed:

  • attenuated intracranial ICA on either side with occluded their cavernous and supraclinoid segments
  • occluded left MCA and attenuated right MCA
  • dilated and extensive lenticulo-striate and thalamo-striate collaterals are seen in the basal ganglia and thalamic regions (puff of smoke)
  • no aneurysmal dilatation or AV malformation
  • normal vertebro-basilar system

Case Discussion

The described features are those of Moyamoya disease with occluded distal ICAs as well as left MCA causing extensive acute left cerebral infarction.

The Ivy sign refers to the leptomeningeal hyper intense FLAIR signal or brighnt sulci due to slow-flowing engorged pial vessels as well as thickened arachnoid membranes.

The Moyamoya means a puff or spiral of smoke describing the cloud-like lenticulostriate and thalamostriate collaterals.

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Case information

rID: 29322
Case created: 16th May 2014
Last edited: 14th Mar 2016
Inclusion in quiz mode: Included

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