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AdrenoleukodystrophyUnusual Computed Tomographic Appearance
Richard S. K. Young, MD;
Jeanette C. Ramer, MD;
Javad Towfighi, MD;
William Weidner, MD;
Ralph Lehman, MD;
Hugo W. Moser, MD
Arch Neurol. 1982;39(12):782-783.
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Adrenoleukodystrophy (ALD) is a disorder in which cerebral demyelination is associated with adrenal insufficiency. It is sex-linked, usually familial, and occurs almost exclusively in males. It is thought to be caused by an abnormal accumulation of saturated long-chain fatty acids in cholesterol esters of the brain and the adrenal glands and in gangliosides.1
The computed tomographic (CT) appearance of ALD has been characterized by symmetry of lesions in the cerebral white matter, late atrophy, variable megaloencephaly, and the absence of mass effect.2-4 We report the clinical, pathologic, and CT findings of a boy with ALD whose CT brain scan initially demonstrated a unilateral low-attenuation lesion in the white matter of the parietal lobe with ventricular distortion suggestive of mass effect. Subsequently, the lesion showed central enhancement with surrounding low attenuation. Later scans disclosed bilateral enhancement in the white matter of the internal and extreme capsules.
REPORT OF
. . . [Full Text PDF of this Article]
Author Affiliations
From the Departments of Pediatrics (Drs Young and Ramer), Pathology (Dr Towfighi), Radiology (Dr Weidner), and Surgery (Dr Lehman), The Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, and the John F. Kennedy Institute for Handicapped Children, Baltimore (Dr Moser).
Footnotes
Accepted for publication Feb 22, 1982.
Reprint requests to Division of Pediatric Neurology, The Milton S. Hershey Medical Center, Hershey, PA 17033 (Dr Young).
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