Moyamoya with leptomeningeal "ivy" sign

Case contributed by Dr Amro Omar

Presentation

Transient ischemic attacks, headache

Patient Data

Age: 13 years
Gender: Female
MRI

2011

FLAIR sequence shows bright sulci or leptomeningeal "ivy" sign, which may be caused by slow-flowing engorged pial vessels, thickened arachnoid membranes

MRI

2013

  • FLAIR: bright sulci or  leptomeningeal "ivy" sign. Given the post-contrast and angiographic findings, this represents slow-flowing engorged pial vessels, thickened arachnoid membranes
  • T1WI C+: leptomeningeal enhancement (contrast-enhanced "ivy" sign)
  • MRA: narrowed distal internal carotid artery and proximal circle of Willis vessels, which would correspond to a "puff of smoke" appearance on conventional angiography

Case Discussion

The ivy sign refers to the MRI appearance of patients with moya moya disease or moya moya syndrome. Prominent leptomeningeal collaterals result in vivid contrast enhancement and high signal on FLAIR due to slow flow. 

Ivy sign differential diagnosis:

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Case information

rID: 32468
Published: 27th Nov 2014
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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