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Related Experiment Videos

Muscle morphometry in amyotrophic lateral sclerosis.

Y Iwasaki1, H Sugimoto, K Ikeda

  • 1Fourth Department of Internal Medicine, Toho University Ohashi Hospital, Tokyo, Japan.

The International Journal of Neuroscience
|June 1, 1991
PubMed
Summary

Amyotrophic lateral sclerosis (ALS) patients show denervation and reinnervation in quadriceps muscles, with some myopathic changes. Muscle fiber preservation, particularly type I and II fibers, appears crucial for ALS progression and severity.

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Area of Science:

  • Neurology
  • Muscle Biology
  • Histopathology

Background:

  • Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons.
  • Muscle pathology in ALS is complex, involving both neurogenic and myogenic components.

Purpose of the Study:

  • To investigate the histopathological and morphometric changes in the quadriceps muscle of ALS patients.
  • To correlate these changes with disease progression and severity.

Main Methods:

  • Quadriceps muscle biopsies were obtained from 24 ALS patients and 15 age-matched controls.
  • Histochemistry and morphometric analysis were performed to assess muscle fiber characteristics.

Main Results:

  • Most ALS patients exhibited pathological features of denervation and reinnervation, with significant inter-patient variability.

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  • Myopathic changes were observed in approximately one-third of ALS cases.
  • Morphometric data indicated a relationship between fiber type (Type I and Type II) duration, diameter, and hypertrophy with disease progression.

    • Conclusions:

    • The findings suggest that both denervation/reinnervation and myopathic changes contribute to ALS pathology.
    • Preservation of both Type I and Type II muscle fibers is important for determining ALS progression and severity.