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PMID: 164848 Published · ppublish English Journal Article Research Support, U.S. Gov't, P.H.S.

Adrenoleukodystrophy. Electron microscopic findings.

Archives of neurology ·Vol. 32 ·No. 4 ·1975-04-00 ·Pages 250-60

Powell H, Tindall R, Schultz P, Paa D, O'Brien J, Lampert P

Abstract

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-Angstrom wide membranes. Similar inclusions were present in adrenocortical cells. These findings and a review of 47 reported cases indicate that adrenoleukodystrophy is a storage disorder caused by a sex-linked recessive error of metabolism.

MeSH Terms

Adrenal Cortex/pathology Adrenal Cortex Function Tests Adrenal Gland Diseases/complications,pathology Adult Autopsy Brain/pathology Brain Diseases/complications,pathology Child Child, Preschool Chromatography, Thin Layer Cytoplasm/ultrastructure Demyelinating Diseases/etiology,pathology Frontal Lobe/ultrastructure Humans Inclusion Bodies/ultrastructure Lymphocytes/pathology Macrophages/pathology Male Microscopy, Electron Organ Size

Authors & Affiliations

6 authors, click to expand affiliations / ORCID

Powell H

Tindall R

Schultz P

Paa D

O'Brien J

Lampert P

Article Info

Journal

Archives of neurology

Abbr.

Arch Neurol

ISSN

0003-9942

DOI

10.1001/archneur.1975.00490460066009

Published

1975-04-00

Pages

250-60

Language

English

Region

United States

NLM ID

0372436

Subset

External Links

PubMed source DOI: 10.1001/archneur.1975.00490460066009 View journal details

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