The ultrastructure of intramuscular nerves in amyotrophic lateral sclerosis
Abstract
Muscle biopsies of 11 patients suffering from amyotrophic lateral sclerosis (ALS) were examined and the i.m. nerves found in seven of them were examined by electron microscopy. In atrophied muscles there was a marked decrease of myelinated fibers. The ultrastructure of the remaining myelinated axons showed changes in the neurofilaments, mitochondria, and vesicles. There was a decrease in the number of unmyelinated fibers as well as the myelinated fibers. Occasionally, there was an increase of unmyelinated fibers containing small fine axons. There were corpora amylacea in unmyelinated axons and banded structures in the extracellular area of the Schwann cells of the unmyelinated fibers. Some of these findings were considered as the ultrastructural features of degeneration and regeneration in i.m. nerves of motoneurons in ALS.
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Accumulations of dysmorphic and enlarged mitochondria, many with deficiencies of critical oxidative enzymes, in anterior horn cells, intramuscular nerves, and skeletal muscle are characteristic of sALS.
“Accumulations of dysmorphic and enlarged mitochondria, many with deficiencies of critical oxidative enzymes, in anterior horn cells, intramuscular nerves, and skeletal muscle are characteristic of sALS [281–285].”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [281]
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References 25 · Cited by 92
- Authors
- Atsumi, T.
- Affiliations
- Jichi Medical University (JP)
- Corresponding author
- Atsumi, T.
- Venue
- Acta Neuropathologica
- Published
- 1981
- Volume
- 55
- Issue
- 3
- Pages
- 193-198
- ISSN
- 0001-6322 (ISSN-L)
- 1432-0533
- OpenAlex
- W1997112145
- Cited by
- 92
- References
- 25
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 7 · 2023: 3 · 2022: 3 · 2021: 7 · 2020: 4 · 2019: 4 · 2018: 3 · 2017: 6 · 2016: 4 · 2015: 6 · 2014: 1 · 2013: 2 · 2012: 6
- FWCI
- 0.52
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Acta Neuropathologica (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Keywords
- amyotrophic lateral sclerosis, intramuscular nerves, nerve ultrastructure, myelinated axons, unmyelinated nerve fibers, corpora amylacea, Schwann cells, neurofilaments, mitochondrial changes, motor neuron degeneration, nerve regeneration, muscle atrophy
- MeSH terms
- Aged, Amyotrophic Lateral Sclerosis, Humans, Middle Aged, Muscles, Nerve Fibers
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