Leukoencephalopathy with swelling in children and adolescents: MRI patterns and differential diagnosis

M. S. van der Knaap, J. Valk, P. G. Barth, L. M.E. Smit, B. G.M. van Engelen, P. Tortori Donati

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57 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)679-686
Number of pages8
Issue number8
Publication statusPublished - Nov 1995


  • Leukoencephalopathy
  • Magnetic resonance imaging
  • White matter swelling

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