Neuropediatrics 2004; 35 - P49
DOI: 10.1055/s-2004-819424

Moya-Moya-syndrome: massive lepto-meningeal enhancement in cMRI mimicking impairment of blood-brain barrier

V Boda 1, S Vlaho 1, S Dittrich 1, L Porto 2, J Gaa 2, P Raikhman 1, M Kieslich 1
  • 1Paediatric Neurology, Johann Wolfgang Goethe University of Frankfurt/Main
  • 2Neuroradiology, Johann Wolfgang Goethe University of Frankfurt/Main, Germany

Objective: Headache and massive leptomeningeal enhanhance in cMRI is very suggestive for infective meningitis. Other causes could be neoplasia and vasculitis. We present another rare differential diagnosis.

Case report: 13 year old boy with bitemporal headache since 6 years and increasing new symptoms with dizziness, nausea, vomiting and visual disturbances without improvement of symptomatic therapy. Because of focal alteration in EEG, cMRI was done, which showed massive leptomeningeal enhancement on FLAIR and contrast-enhanced T1-weighted MR images. Spinal fluid studies were normal. Then MR-angiography showed stenosis in ACI on both sides. The diagnosis of Moya-Moya disease was made by conventional angiography.

Discussion: Contrast-enhanced T1-weighted MR images does not necessarily mean impairment of blood-brain-barrier. In Moya-Moya disease the reason for contrast-enhancement is hyperperfusion due to massive development of collateral vessels. In literature the enhancement on FLAIR-weighted MR images is discribed as „ivy-sign“.

Conclusions: Moya-Moya disease can be the reason for unspecific headache, leptomeningeal enhancement and normal spinal fluid.

Keywords: Moya Moya, leptomeningeal enhancement, Ivy-Sign