ADOLESCENT CASE OF ALEXANDER-DISEASE - MR-IMAGING AND MR SPECTROSCOPY

A 17-year-old male was diagnosed as having Alexander disease from the clinical manifestations (psychomotor deterioration and megalencephaly) , neuroradiologic findings (frontal dominant leukodystrophy), and elev ation of alpha B-crystallin and heat shock protein 27 in the cerebrosp inal fluid, He exhibited increased attenuation on computed tomography and T-1 and T-2 shortening on magnetic resonance imaging in the bilate ral basal ganglia and thalamus. Some paramagnetic substances might be deposited in the basal ganglia and thalamus in the late stage of Alexa nder disease, at least 8 years after onset. (C) 1998 by Elsevier Scien ce Inc. All rights reserved.