J Korean Radiol Soc.  1994 Jul;31(1):171-176.

MR Imaging of Childhood Adrenoleukodystrophy

• Kim TK
• Kim IO
• Kim WS
• Yeon KM

Abstract

PURPOSE
The purpose of this study was to analyze the MR findings of childhood adrenoleukodystrophy regarding the extent of white matter degeneration and the pattern of enhancement, and to correlate these findings with clinical manifestations.
MATERIALS AND METHODS
Six male patients (5-13 years old;mean age, 8.7 years) of biochemically confirmed adrenoleukodystrophy 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 pattern of enhancement. Also we correlated the MR findings with clinical manifestations of symptoms and the results of evoked potential studies.
RESULTS
The most common sites of predominant involvement were occipital deep white matter, optic radiation, and splenium of corpus callosum (n = 4, 66.7%). The other sites of predominant involvement were frontal deep white matter (n = 1, 16.7%) and pyramidal tract with cerebellum (n = 1, 16.7%). After contrast enhancement, there was peripheral rim enhancement surrounding the most severely affected areas in five cases (83. 3%). These findings were consistent with clinical symptoms in five cases (83.3%), but the results of evoked potential studies were consistent with clinical symptoms in only two cases (33.3%).
CONCLUSION
In childhood adrenoleukodystrophy, MRI can clearly demonstrate specific tracts of involvement and these findings were well correlated with clinical manifestations.

MeSH Terms

Adrenoleukodystrophy*
Cerebellum
Corpus Callosum
Evoked Potentials
Humans
Magnetic Resonance Imaging*
Male
Pyramidal Tracts