Alexander's disease - MedPix® Medical Image Database and Teaching Files

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Disease Topic 1491
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Contributor: Val M. Runge, MD

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Alexander's disease
Factoid 1491 - Created: 2001-03-26 12:45:00-05 - Modified: 2001-09-25 15:50:30-04

ACR Codes: 1.-1
Alexander's disease (AD) is a rare dysmyelinating leukodystrophy. Males are most often affected, and the disease appears to occur sporadically without a familial incidence. AD can be divided into three clinical subtypes: infantile, juvenile, and adult. The infantile type is most common with onset at birth to early childhood. Characteristic clinical features include enlarging head size, psychomotor retardation, spastic quadraparesis, and seizures. The primary metabolic defect of AD is not known, but an error in astrocytic metabolism is assumed. Neuropathologic reports describe an accumulation of eosinophilic deposits in astrocytes in younger patients and Rosenthal fibers in older children and adults. Radiologic descriptions relate a greater degree of involvement of the frontal and temporal lobe white matter. There is involvement of the subcortical arcuate fibers. Enhancement on computerized tomography has been reported in early stages of the disease.

Location: Brain and Neuro	Sublocation: none selected	Category: Congenital, genetic
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Written by: Val M. Runge, MD
Prepared by: Neuroradiology Learning File - © ACR
Affiliation: ACR Learning File® - || -
Approved by: James G. Smirniotopoulos, M.D.
Affiliation: Uniformed Services University - || -

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