Adrenoleukodystrophy: Electron Microscopic Findings
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.
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Spicules are straight and curved striated structures made of degenerated myelin, also observed in 15% of Schwann cells surrounding myelinated axons in the adrenals.
“Spicules are straight and curved striated structures made of degenerated myelin [17,20,22,74,75,76,77,78,79,80], which are also observed in 15% of Schwann cells surrounding myelinated axons in the adrenals [19,81].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [19]
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Adrenoleucodystrophie de l'enfant et adrenomyeloneuropathie. Etude de deux familles
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An autopsy case of atypical adrenoleukomyeloneuropathy in childhood
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Adrenoleukodystrophy with involvement of the cerebral cortex
Kakita, A., Ishikawa, A., Koike, R., Tsuji, S., & Takahashi, H. (1997) · Neuropathology · doi:10.1111/j.1440-1789.1997.tb00022.x · Cited by 1 · see citing works
Neurophysiological investigations in two cases of Alexander's disease with infantile onset
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Genetic and Metabolic Diseases
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- Authors
- Powell, H.
- Corresponding author
- Powell, Henry
- Venue
- Archives of Neurology
- Publisher
- American Medical Association (AMA)
- Published
- 1975-04-01
- Volume
- 32
- Issue
- 4
- Pages
- 250
- ISSN
- 0003-9942 (ISSN-L)
- OpenAlex
- W2011989937
- Cited by
- 76
- References
- 46
- Citations by year
- 2025: 1 · 2024: 1 · 2020: 1 · 2018: 1
- FWCI
- 3.89
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Archives of Neurology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Health Sciences › Medicine › Rheumatology › Glycogen Storage Diseases and Myoclonus
- Keywords
- adrenoleukodystrophy, ultrastructure, neurochemistry, glial cells, cytoplasmic inclusions, adrenocortical cells, storage disorders, X-linked recessive inheritance, white matter
- MeSH terms
- Adrenal Cortex, Adrenal Cortex Function Tests, Adrenal Gland Diseases, Adult, Autopsy, Brain, Brain Diseases, Inclusion Bodies, Child, Child, Preschool, Chromatography, Thin Layer, Cytoplasm, Demyelinating Diseases, Frontal Lobe, Humans, Lymphocytes, Macrophages, Male, Microscopy, Electron, Organ Size
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