Adrenomyeloneuropathy: Report of a family and electron microscopical findings in peripheral nerve
Abstract
A family with adrenomyeloneuropathy (AMN) is reported, whose female carriers showed severe neurological and biochemical abnormalities. Cytoplasmic lamellar inclusions were found in endoneurial cells of the biopsied sural nerve of a male patient. In spite of the peripheral nervous system involvement in patients with AMN, there have been few reports of these inclusions in the peripheral nerves of such cases.
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Sural nerves in AMN suffer more specific involvement of large myelinated fibers, causing deficits in position and vibratory sensations.
“Sural nerves suffer more specific involvement of large myelinated fibers [87], causing deficits in position and vibratory sensations.”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [87]
Cites
Patterns of copulatory behavior in male mammals
Dewsbury, D. A. (1972) · The Quarterly Review of Biology · doi:10.1086/407097 · Cited by 330 · see citing works
Adrenomyeloneuropathy: A report on two families
Martin, J. J., Lowenthal, A., Ceuterick, C., & Gacoms, H. (1982) · Journal of Neurology · doi:10.1007/bf00313395 · Cited by 27 · see citing works
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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
Nerve conduction studies in adrenomyeloneuropathy
Chaudhry, V, Moser, H. W., & Cornblath, D. R. (1996) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp.61.2.181 · Cited by 73 · 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
- Tanaka, K., Koyama, A., Koike, R., Ohno, T., Atsumi, T., & Miyatake, T.
- Affiliations
- Brain Research Institute, Niigata University (JP)
- ORCID
- Koyama, A.
- Venue
- Journal of Neurology
- Published
- 1985-05
- Volume
- 232
- Issue
- 2
- Pages
- 73-78
- ISSN
- 0012-1037 (ISSN-L)
- 0340-5354
- 1432-1459
- OpenAlex
- W2088859275
- Cited by
- 18
- References
- 2
- Citations by year
- 2025: 2 · 2022: 1 · 2014: 1
- FWCI
- 0.57
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Journal of 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 › Physiology › Lysosomal Storage Disorders Research
- Keywords
- adrenomyeloneuropathy, familial inheritance, female carriers, peripheral nerve involvement, sural nerve, electron microscopy, neurological abnormalities, biochemical abnormalities
- MeSH terms
- Adrenal Gland Diseases, Adult, Aged, Biopsy, Carrier State, Demyelinating Diseases, Female, Humans, Male, Microscopy, Electron, Muscle Spasticity, Paralysis, Pedigree, Spinal Nerves, Sural Nerve
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