MRI and CT findings in Krabbe disease

Pediatr Neurol. 1991 Jul-Aug;7(4):283-8. doi: 10.1016/0887-8994(91)90046-n.


The progression and characteristics of magnetic resonance imaging (MRI) and computed tomographic (CT) findings in 3 patients with infantile Krabbe disease (i.e., globoid cell leukodystrophy or galactocerebroside beta-galactosidase deficiency) are reported. We obtained initial CT and MRI studies when patients demonstrated hyperirritability and hypertonicity. The following results facilitated early diagnoses: increased density in the thalami, corona radiata, and cerebellar cortex on CT and plaque-like, high signal intensity in the periventricular region and cerebellar white matter on MRI T2-weighted images. After severe motor and mental deterioration and spasticity had developed, progressive brain atrophy, low density in the white matter, and calcification-like, symmetric, punctate high-density areas in the corona radiata were evident on CT and high signal intensity in T2-weighted images and low signal intensity in T1-weighted images in the white matter were present on MRI. In particular, linear patterns were observed in the centrum semiovale on MRI.

Publication types

  • Case Reports

MeSH terms

  • Brain / diagnostic imaging*
  • Brain / pathology*
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Leukodystrophy, Globoid Cell / diagnostic imaging*
  • Leukodystrophy, Globoid Cell / pathology*
  • Magnetic Resonance Imaging
  • Tomography, X-Ray Computed