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.
Sasaki
M
M
Division of Child Neurology, National Center Hospital for Mental Nervous and Muscular Disorder, Tokyo, Japan.
Sakuragawa
N
N
Takashima
S
S
Hanaoka
S
S
Arima
M
M
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