Hypomyelination with T2-hypointense globi pallidi in a child with fucosidosis

J Child Neurol. 2014 Jul;29(7):988-9. doi: 10.1177/0883073813488667. Epub 2013 May 10.

Abstract

A 4-year-old boy presented with progressive neurodegeneration, mild coarsening of facies and spasticity. The classical neuroimaging guided the subsequent investigation of enzyme assay which confirmed the diagnosis of fucosidosis.

Keywords: dysostosis; fucosidosis; leukodystrophy; lysosomal storage disorders.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Demyelinating Diseases / complications*
  • Fucosidosis / complications*
  • Fucosidosis / pathology*
  • Globus Pallidus / pathology*
  • Humans
  • Magnetic Resonance Imaging, Cine
  • Male