MRI and CT findings in Krabbe disease.
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.
Division of Child Neurology, National Center Hospital for Mental Nervous and Muscular Disorder, Tokyo, Japan.
M
M
Sasaki
N
N
Sakuragawa
S
S
Takashima
S
S
Hanaoka
M
M
Arima
D001921:Brain / Q000000981:diagnostic imaging* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007965:Leukodystrophy, Globoid Cell / Q000000981:diagnostic imaging* / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D014057:Tomography, X-Ray Computed