J Korean Radiol Soc.  1995 Sep;33(3):433-437.

MR Imaging of Childhood Metachromatic Leukodystrophy

• Choi YS
• Lee JY
• Kim TS
• Kim IO
• Yeon KM
• Kim OH
• Hwang YS

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.

MeSH Terms

Brain
Corpus Callosum
Diagnosis, Differential
Extremities
Female
Humans
Leukodystrophy, Metachromatic*
Magnetic Resonance Imaging*
Pyramidal Tracts