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Cited by 36Adrenal Hormones and DisordersMetabolism and Genetic DisordersHereditary Neurological Disorders

Adrenomyeloneuropathy: A protracted, pseudosystematic variant of adrenoleukodystrophy

Probst, A., Ulrich, J., Heitz, Ph. U., Herschkowitz, N.·1980·Acta Neuropathologica
DOI 10.1007/bf00690750
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Abstract

Histopathological, immunocytochemical, and electron microscopical investigations were carried out in a man with a protracted history of spastic paraparesis, adrenal insufficiency and hypogonadism. Pathological findings were identical with those of the few previously reported cases of adrenomyeloneuropathy (AMN) including cytoplasmic lamellar inclusions consisting of two parallel 2.5 nm leaflets separated by a clear space of variable extent, in the brain, spinal roots, adrenal gland, and interstitial cells of the testis. No inclusions could be found in oligodendrocytes. In brain macrophages they are thought to represent breakdown products of pathological myelin stored in lysosomes, whereas in other localizations they might be an expression of the primary metabolic defect of the cell. Special attention was paid to the pseudosystematic type of fiber tract degeneration in the spinal cord. The dying-back pattern of axonal destruction was interpreted as a possible result of the multisegmental demyelination observed in these tracts. All known hormones could be localized in the pituitary by immunocytochemistry. Corticotrophs and gonadotrophs were numerous. The structural damage of the adrenal cortex and the interstitial cells of the testis is, therefore, considered to result from the inborn error of metabolism on the one hand and from an enhanced stimulation exerted by ACTH and gonadotrophins on the other.

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Crossref · 10.1007/bf00690750

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  • In AMN patients, the fasciculus gracilis shows a prominent loss of myelinated fibers and oligodendrocytes, associated with axonal atrophy and very thin or disintegrating myelin sheaths.

    “In AMN patients, the fasciculus gracilis shows a prominent loss of myelinated fibers and OLs [18], associated with axonal atrophy and very thin or disintegrating myelin sheaths [49,82].”

    From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [82]

  • Cuffs of perivascular macrophages are common in the degenerating tracts in AMN.

    “Cuffs of perivascular macrophages are common in the degenerating tracts [49,82].”

    From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [82]

  • 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 [82]

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Authors
Probst, A., Ulrich, J., Heitz, P. U., & Herschkowitz, N.
Affiliations
  • University of Basel (CH)
  • University of Bern (CH)
Venue
Acta Neuropathologica
Publisher
Springer Science and Business Media LLC
Published
1980
Volume
49
Issue
2
Pages
105-115
ISSN
  • 0001-6322 (ISSN-L)
  • 1432-0533
DOI
10.1007/bf00690750
OpenAlex
W2316607880
Cited by
36
References
26
Citations by year
2025: 2 · 2024: 6 · 2022: 1 · 2021: 1 · 2016: 1 · 2012: 1
FWCI
3.05
Type
journal article
Language
en
Open access
closed
Licence
www.springer.com/tdm (tdm)
Locations
  • Acta Neuropathologica (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Keywords
adrenomyeloneuropathy, spastic paraparesis, adrenal insufficiency, hypogonadism, lamellar inclusions, lysosomes, demyelination, dying-back degeneration, ACTH stimulation test, gonadotropins, testicular interstitial cells, inborn errors of metabolism
MeSH terms
Adrenal Insufficiency, Adrenal Glands, Adult, Brain, Inclusion Bodies, Diffuse Cerebral Sclerosis of Schilder, Humans, Hypogonadism, Male, Microscopy, Electron, Oligodendroglia, Spinal Cord, Spinal Nerve Roots, Testis

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