MR imaging and 1H-MR spectroscopy in a case of juvenile Alexander disease

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.