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April 1975

Adrenoleukodystrophy: Electron Microscopic Findings

Author Affiliations

From the Department of Neurosciences, University Hospital and School of Medicine, University of California at San Diego (Drs. Tindall and O'Brien), Childrens Hospital of San Diego (Drs. Schultz and Paa), and Department of Pathology, Veterans Hospital and the University of California at San Diego (Drs. Powell and Lampert).

Arch Neurol. 1975;32(4):250-260. doi:10.1001/archneur.1975.00490460066009

Ultrastructural and neurochemical studies were done on three male patients with adrenoleukodystrophy. In each case, the affected white matter contained enlarged glial cells filled with pathognomic intracytoplasmic inclusions consisting of electron-lucent spicules bounded by 25-Angström wide membranes. Similar inclusions were present in adrenocortical cells. These findings and a review of 47 reported cases indicate that adrenoleu-kodystrophy is a storage disorder caused by a sex-linked recessive error of metabolism.

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