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Leukoencephalopathy with swelling in children and adolescents: MRI patterns and differential diagnosis
Authors:M S van der Knaap  J Valk  P G Barth  L M E Smit  B G M van Engelen  P Tortori Donati
Institution:(1) Department of Child Neurology, Free University Hospital, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands;(2) Department of Diagnostic Radiology, Free University Hospital, Amsterdam, The Netherlands;(3) Department of Child Neurology, Academic Medical Center, Amsterdam, The Netherlands;(4) Department of Neurology, Sint Radboud University Hospital, Nijmegen, The Netherlands;(5) Department of Paediatric Neuroradiology, Giannina Gaslini Scientific Institute, Children's Hospital, Genoa, Italy
Abstract:In children, several neurological disorders are characterised by spongiform leukoencephalopathy. MRI of the brain typically shows white matter swelling, but does not enable differentiation of the various underlying disorders. The aim of this article is optimisation of the diagnostic value of MRI in leukoencephalopathy accompanied by swelling. MRI-based inclusion criteria were met by 20 patients in our database. The images were analysed using a detailed scoring list. In 13 of the 20 patients the clinical diagnosis was known (11 definite and 2 probable diagnoses). Characteristic MRI abnormalities could be defined in these patients. Of the 7 patients without a diagnosis, 5 had identical MRI abnormalities: difuse hemisphere swelling and typical cysts in frontoparietal subcortical white matter and the tips of the temporal lobes. The clinical picture was also similar in these patients, suggesting a similar disease.
Keywords:Leukoencephalopathy  White matter swelling  Magnetic resonance imaging
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