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.

journal_name

Pediatr Neurol

journal_title

Pediatric neurology

authors

Sasaki M,Sakuragawa N,Takashima S,Hanaoka S,Arima M

doi

10.1016/0887-8994(91)90046-n

subject

Has Abstract

pub_date

1991-07-01 00:00:00

pages

283-8

issue

4

eissn

0887-8994

issn

1873-5150

pii

0887-8994(91)90046-N

journal_volume

7

pub_type

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