MR Imaging of Childhood Metachromatic Leukodystrophy.
10.3348/jkrs.1995.33.3.433
- Author:
Yun Sun CHOI
;
Jae Young LEE
;
Tae Sung KIM
;
In One KIM
;
Kyung Mo YEON
;
Ok Hwa KIM
;
Yong Seung HWANG
- Publication Type:Original Article
- MeSH:
Brain;
Corpus Callosum;
Diagnosis, Differential;
Extremities;
Female;
Humans;
Leukodystrophy, Metachromatic*;
Magnetic Resonance Imaging*;
Pyramidal Tracts
- From:Journal of the Korean Radiological Society
1995;33(3):433-437
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: The purpose of this study was to analyze the characteristic MR findings of childhood metachromatic leukodystrophy. MATERIALS AND METHODS: Five female patients (10--29 months old;mean age, 21.8 months) of biochemically confirmed metachromatic leukodystrophy were included in this study. We evaluated the extent of white matter degeneration, which was shown as high signal intensity on T2-weighted image, and the presence or absence of the enhancement. Result.' All 5 cases showed high signal intensity in periventricular deep white matter and centrum semiovale which were bilateral, symmetric and confluent. Posterior predominace, sparing of subcortical U fibers and immediate periventricular white matter, and the involvement of splenium of corpus callosum were also noted in all cases. There were other manifestations, such as 'tigroid pattern' in centrum semiovale (n=4), the involvement of genu of corpus callosum(n=4), posterior limb of internal capsule(n=4), descending pyramidal tracts (n=3), deep cerebellar white matter(n=1), claustrum(n=2), and diffuse brain atrophy(n=1). In three cases with Gd-infusion, contrast enhancement of the lesion was not seen. CONCLUSION: In childhood metachromatic leukodystrophy, MRI can clearly demonstrate the chracteristic extent of the white matter lesion and other associated findings, facilitating the differential diagnosis from other similar leukodystrophies.
