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Related Experiment Videos

Amyotrophic lateral sclerosis: current issues in classification, pathogenesis and molecular pathology

P G Ince1, J Lowe, P J Shaw

  • 1Department of Neuropathology, Newcastle General Hospital, University of Newcastle upon Tyne, UK.

Neuropathology and Applied Neurobiology
|June 20, 1998
PubMed
Summary

Amyotrophic lateral sclerosis (ALS) classification may need revision. Related motor neuron disorders might share underlying causes with ALS, suggesting a spectrum of disease presentation.

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Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Current amyotrophic lateral sclerosis (ALS) classification relies on El Escorial criteria.
  • These criteria may exclude related disorders with shared etiological mechanisms from research and clinical trials.
  • Understanding the spectrum of motor neuron diseases is crucial for comprehensive diagnosis and treatment.

Purpose of the Study:

  • To reconsider the classification of ALS in light of molecular and histopathological advancements.
  • To explore the relationship between ALS and other motor neuron disorders, including progressive lateral sclerosis, progressive muscular atrophy, and ALS-dementia.
  • To propose a clinicopathological spectrum unifying these conditions.

Main Methods:

  • Review of current literature on molecular pathogenesis and histopathology of ALS and related disorders.

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  • Analysis of the El Escorial criteria's impact on clinical trial eligibility.
  • Comparison of molecular pathology, including inclusion bodies, across different syndromes.
  • Examination of lesion distribution and its effect on clinical phenotype.

    • Main Results:

    • Evidence suggests a shared pathogenetic cascade underlies ALS and related motor neuron disorders.
    • Oxidative stress and glutamatergic dysfunction appear relevant across familial and sporadic ALS.
    • Similarities in inclusion body pathology (ubiquitinated and hyaline conglomerate) are observed.
    • Anatomical lesion distribution dictates the specific motor and cognitive features.

    Conclusions:

    • ALS and related disorders may represent a clinicopathological spectrum, not distinct entities.
    • This spectrum concept, driven by pathogenetic similarities and variable lesion distribution, offers a new framework.
    • This perspective is relevant to other neurodegenerative diseases and will inform future therapeutic strategies targeting underlying mechanisms.