Adreno-leukodystrophy

Abstract

Ultrastructural observations of an adrenal biopsy specimen from an 11-year-old boy with adreno-leukodystrophy showed that adrenocortical cells contained cytoplasmic accumulations of lamellar material. Each lamella consisted of two parallel, electron-dense 25 Angstrom leaflets separated by a variable 20 A to 70 A space. Rows of glycogen and a clear space often surrounded this material. Similar accumulations were also present in the Schwann cell cytoplasm of many of the myelinated nerve fibers in the adrenal capsule. These findings represent a possible morphologic link between the endocrine and nervous systems in this disease.