Abstract
The serial MR image and MR spectroscopy in the brain were examined in a young male
diagnosed as having juvenile Alexander disease. He had megalencephaly, psychomotor
retardation, seizures, and increasing elevation of increasing alpha-B crystallin and
heat shock protein 27 in the cerebrospinal fluid. Serial MR images demonstrated increased
demyelination of the bilateral frontal region to left occipital region over several
years. The myo-inositol/creatine ratio was significantly increased in both the demyelinated white
matter and normal area in the MR spectroscopy. These results suggested that demyelination
very slowly progressed from the frontal to occipital region and that glial degeneration
may occur even in the unaffected white matter of patients with juvenile Alexander
disease.
Keywords
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Article info
Publication history
Accepted:
May 16,
2002
Received in revised form:
May 11,
2002
Received:
December 20,
2001
Identification
Copyright
© 2002 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
