Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis

Asao Hirano, Imaharu Nakano, Leonard T. Kurland, Donald W. Mulder, Paul W. Holley, Geno Saccomanno

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Abstract

Lewy body-like hyaline inclusions in the soma and swollen, cord-like cell processes are characteristic alterations of the anterior horn cells in familial amyotrophic lateral sclerosis (ALS) with posterior column and spinocerebellar tract involvement. A fine structural analysis of these two structures has been performed in two brothers from a family (“C” family) previously described by Kurland and Mulder in 1955. The perikaryal hyaline inclusions consisted of accumulations of randomly oriented neurofilaments interspersed with thick linear densities associated with granular material. Some of the accumulations showed a central condensation. Cord-like, swollen neuronal processes were composed, for the most part, of numerous neurofilaments arranged parallel to the long axes. Dense structures were sometimes observed within the large bundles of filaments. They were composed of ill-defined dense, granular and fibrillar material associated with scattered vesicles and mitochondria. These dense areas were sometimes surrounded by various amounts of fine filaments, approximately 5 nm in diameter.

Original languageEnglish (US)
Pages (from-to)471-480
Number of pages10
JournalJournal of Neuropathology and Experimental Neurology
Volume43
Issue number5
DOIs
Publication statusPublished - Sep 1984

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Keywords

  • Anterior horn cells
  • Familial amyotrophic lateral sclerosis
  • Hyaline neuronal inclusions
  • Neurofilaments

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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