Krabbe disease is characterized by abnormal breakdown and turnover of
myelin, leading to extensive demyelination in both the peripheral and
central nervous systems. A 7-month-old infant with early-onset Krabbe
disease had deceptively normal head images, but spinal MRI demonstrate
d abnormal gadolinium enhancement of the lumbosacral sacral nerve root
s and cauda equina such as that seen in Guillain-Barre syndrome. Abnor
mal enhancement in spinal MRI has not been previously described in pat
ients with leukodystrophies, (C) 1998 by Elsevier Science Inc. All rig
hts reserved.