Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis

Walter G Bradley, P. Good, C. G. Rasool, L. S. Adelman

Research output: Contribution to journalArticle

183 Citations (Scopus)

Abstract

Phrenic nerves of 11 patients with amyotrophic lateral sclerosis studied postmortem contained only 33% of the normal number of large myelinated fibers (9 controls; p < 0.001). In the phrenic nerves of these patients, there were 18% fewer large myelinated fibers in the distal segment than in the proximal segment (p < 0.025). The ratio of axonal circumference to myelin lamellae in large myelinated fibers in the distal segment was 34% greater than that in control fibers (p < 0.002). The proportion of acute axonal degeneration was the same at all levels (48.0 ± 13.7%). Sural nerves of 21 patients with amyotrophic lateral sclerosis had more acute axonal degeneration and 30% fewer myelinated fibers (p < 0.05) than controls; evidence of degeneration also extended to unmyelinated fibers. The amount of axonal transport of acetylcholinesterase in 9 sural nerves determined in vitro was reduced by 24% (p < 0.05) and the apparent transport rate was reduced by 44% (p < 0.01) compared with 4 controls. These findings show that in amyotrophic lateral sclerosis a small degree of dying-back change and of distal axonal atrophy is superimposed on the degradation of motor neuron cell bodies, and that the disease effects spread beyond the motor neurons.

Original languageEnglish
Pages (from-to)267-277
Number of pages11
JournalAnnals of Neurology
Volume14
Issue number3
StatePublished - Jan 1 1983
Externally publishedYes

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Amyotrophic Lateral Sclerosis
Peripheral Nerves
Sural Nerve
Phrenic Nerve
Motor Neurons
Axonal Transport
Acetylcholinesterase
Myelin Sheath
Atrophy

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis. / Bradley, Walter G; Good, P.; Rasool, C. G.; Adelman, L. S.

In: Annals of Neurology, Vol. 14, No. 3, 01.01.1983, p. 267-277.

Research output: Contribution to journalArticle

Bradley, Walter G ; Good, P. ; Rasool, C. G. ; Adelman, L. S. / Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis. In: Annals of Neurology. 1983 ; Vol. 14, No. 3. pp. 267-277.
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