Moyamoya with recurrent cerebral infarctions

Case contributed by Dr Mohammad A. ElBeialy

Presentation

11 months old baby with recurrent strokes presented initially with right sided hypotonia, feeding problems and delayed developmental milestones.The patient underwent non-contrast CT. Three months later, the patient developed left sided hemiplegia and underwent MRI brain.

Patient Data

Age: 11 months
Gender: Male

A patchy cortical and sub-cortical ill-defined hypodensity is seen within the left high frontal lobe with effacement of the related cortical sulci.

Findings:

  • A large right fronto-parietal and anterior temporal cortical and subcortical area of high T2 signal intensity with restricted diffusion demonstrating high DWI signal. Associated cytotoxic brain edema is seen with effacement of the related cortical sulci, mild compression of the left lateral ventricle and slight contralateral midline shift.
  • A left fronto-parietal deep white matter patchy area of high T2/FLAIR signal with slight exvacuuo-dilatation of the left lateral ventricle. The left cerebral hemisphere appears relatively small sized. Patchy peri-ventricular white matter areas of bright T2/FLAIR signals are noted.
  • Axial T2 images shows attenuation of the distal ICA on either side.
  • No hydrocephalic changes.
  • Normal posterior fossa.
  • Left mastoiditis with opacification and T2 prolongation is incidentally noted.

Conclusion:

  • A large right fronto-parietal and anterior temporal area of restricted diffusion with cytotoxic brain edema; representing acute infarction.

  • Left high fronto-parietal old infarction and periventricular leukomalacia with consequent reduction of the left cerebral hemisphere.

  • The recurrent bilateral cerebral infarctions and the possible attenuation of the distal ICA could suggest idiopathic progressive arteriopathy of childhood or Moyamoya disease; for MSCT or MR cerebral angiography.

     

  • Marked progressive attenuation of the distal ICAs; namely the right cavernous segment and the supra-clinoid segment of either ICA, together with their bifurcations, and both A1 and M1 segments of the ACA and MCA respectively as well as attenuation of the proximal circle of willis.
  • Hypertrophied lenticulostriate and thalamostriate arteries with multiple basal ganglia collaterals. Contrast-enhanced CT shows enhancing dots within the basal ganglia and abnormal enhancing network of vessels at the base of the brain.
  • Fairly patent distal anterior cerebral and middle cerebral arteries on both sides.
  • Persistent fetal origin of both PCAs with normal caliber as well as the basilar artery and scanned distal vertebral arteries.
  • No evidence of cerebral aneurysms or arterio-venous malformations.
  • Normal appearance of the dural venous sinuses and cerebral veins.
  • Global brain atrophy with reduced volume of the right cerebral hemisphere.

Case Discussion

This case shows typical Moyamoya disease with recurrent bilateral cerberal infarctions and brain atrophy. CT angiography shows progressive attenuation of the distal ICAs and the proximal circle of Willis, with collateralization through hypertrophied lenticulo-striate and thalamo-striate arteries. The word Moyamoya is a Japanese word that means a puff or spiral of smoke describing the  cloud-like lenticulostriate and thalamostriate collaterals on conventional or CT angiography.

 

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Case information

rID: 29207
Case created: 9th May 2014
Last edited: 16th Dec 2015
Inclusion in quiz mode: Included

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