MRI and CT findings in Krabbe disease.

Abstract

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.

• Authors: M Sasaki, N Sakuragawa, S Takashima, S Hanaoka, M Arima
• Journal: Pediatric neurology (Pediatr Neurol) 1991 Jul-Aug Vol. 7 Issue 4 Pg. 283-8 ISSN: 0887-8994 [Print] United States
• PMID: 1930420 (Publication Type: Case Reports, Journal Article)

Topics

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