Adrenomyeloneuropathy: Demonstration of inclusions at the level of the peripheral nerve
Abstract
A patient is reported with a progressive neurological disease which started at the age of 19 years and included spasmodic paraplegia, urinary disorders, impotence and neuropathic signs. The neurological syndrome was associated with adrenal failure. The disease led to death within 5 years. In this characteristic picture of adrenomyeloneuropathy, biopsy of peripheral nerve disclosed lamellar inclusions in the Schwann cell cytoplasm. Such structures, which have already been observed in the sural nerve of some adrenolcukodystrophy cases reported previously, are described for the first time in a case of adrenomyeloneuropathy.
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Peripheral nerves in AMN show variable and mild lesions of chronic axonal atrophy with secondary demyelination.
“The few studies on peripheral nerves in AMN [16,18,20,82,84,85,86] highlight variable and mild lesions of chronic axonal atrophy with secondary demyelination [49].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [86]
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A Two-Year Trial of Oleic and Erucic Acids (“Lorenzo's Oil”) as Treatment for Adrenomyeloneuropathy
Aubourg, P., Adamsbaum, C., Lavallard-Rousseau, M.-C., Rocchiccioli, F., Cartier, N., Jambaque, I., Jakobezak, C., Lemaitre, A., Boureau, F., Wolf, C., et al. (1993) · New England Journal of Medicine · doi:10.1056/nejm199309093291101 · Cited by 242 · see citing works
Adrenomyeloneuropathy: A Neuropathologic Review Featuring Its Noninflammatory Myelopathy
Powers, J. M., DeCiero, D. P., Ito, M., Moser, A. B., & Moser, H. W. (2000) · Journal of Neuropathology & Experimental Neurology · doi:10.1093/jnen/59.2.89 · Cited by 204 · see citing works
Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy
Bougnères, P., & Le Stunff, C. (2025) · Genes · doi:10.3390/genes16050590 · Cited by 11 · see citing works
- Authors
- Julien, J., Vallat, J. M., Vital, C., Lagueny, A., Ferrer, X., & Darriet, D.
- Affiliations
- Hôpital Pellegrin (FR)
- ORCID
- Ferrer, X.
- Venue
- European Neurology
- Publisher
- S. Karger AG
- Published
- 2008-01-29
- Volume
- 20
- Issue
- 5
- Pages
- 367-373
- ISSN
- 0014-3022 (ISSN-L)
- 1421-9913
- OpenAlex
- W2061127621
- Cited by
- 19
- References
- 0
- Citations by year
- 2025: 2 · 2024: 1 · 2022: 1 · 2014: 2 · 2012: 1
- FWCI
- 0.00
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- European Neurology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
- Keywords
- adrenomyeloneuropathy, peripheral nerve biopsy, Schwann cells, lamellar inclusions, adrenal insufficiency, paraplegia, neurogenic bladder, erectile dysfunction, sural nerve
- MeSH terms
- Adrenal Insufficiency, Adult, Biopsy, Inclusion Bodies, Cytoplasm, Humans, Macrophages, Male, Microscopy, Electron, Paraplegia, Peroneal Nerve, Schwann Cells
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