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The Ivy Sign in Adult MoyaMoya Disease

A 30 years old gentleman with no known medical illness presented with headache and vomiting associated with right hemiparesis. Neurological examination revealed reduced power of both lower limbs (4/5). The patient’s condition deteriorated, and he subsequently passed away. “Ivy sign” was first described in 1995, so called because of its resemblance to ‘ivy creeping on stone’(1) noted in post contrast MR images of approximately 70% of Moyamoya patients (2). It appears as continuous or discontinuous linear leptomeningeal enhancement due to accumulation of gadolinium in engorged pial vascular network formation that causes leptomeningeal collateral development (1, 2). It can also be depicted on FLAIR image since fluid signal is effectively suppressed in this sequence; appears as high signal intensities along the cortical sulci and subarachnoid spaces (3). Recognizing Ivy sign as part of Moyamoya disease will avoid misdiagnosis, confusion with other differential diagnosis and eliminate potential unnecessary investigations.