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Progressive Spastic Paraparesis and Adrenal Insufficiency
Maria Gumbinas, MD;
H. Mei Liu, MD;
Glyn Dawson, MD;
Marianne Larsen, MD;
Orville Green, MD
Arch Neurol. 1976;33(10):678-680.
Abstract
• A 10-year-old boy with progressive paraparesis, personality change, and seizures had laboratory evidence of adrenal insufficiency. Pathologic study showed cerebral edema, but no loss of myelin. Notable pathologic changes were limited to the spinal cord, where the corticospinal and spinocerebellar tracts were demyelinated. Lipid analysis of the brain was normal apart from the finding that galactocerebroside contained a higher proportion than normal of  -hydroxy fatty acids. We suggest that this case represents a distinct disease, differing importantly from adrenoleukodystrophy. The underlying defect appears to be in the early enzymatic pathway before cholesterol synthesis, although it is also possible that the defect is at the cell membrane.
Author Affiliations
From the departments of pediatrics and neurology, University of Maryland Medical School, Baltimore (Dr Gumbinas), the departments of pediatrics, neurology, and pathology, Northwestern University Medical School, and the Children's Memorial Hospital (Drs Liu, Larsen, and Green), and the departments of pediatrics and biochemistry, University of Chicago, Pritzker School of Medicine (Dr Dawson), Chicago.
Footnotes
Accepted for publication Jan 16, 1976.
Reprint requests to Department of Pediatrics, University of Maryland Hospital, 22 S Greene St, Baltimore, MD 21201 (Dr Gumbinas).
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