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Updated: Jan 27, 2026

Eye-Tracking Control to Assess Cognitive Functions in Patients with Amyotrophic Lateral Sclerosis
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Defining pre-symptomatic amyotrophic lateral sclerosis.

Michael Benatar1, Martin R Turner2, Joanne Wuu1

  • 1a Department of Neurology , University of Miami , Miami , FL , USA and.

Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration
|March 21, 2019
PubMed
Summary
This summary is machine-generated.

Understanding pre-symptomatic amyotrophic lateral sclerosis (ALS) is key for early intervention. A new model proposes two stages of pre-symptomatic ALS: pre-manifest and prodromal, aiding therapeutic development.

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Area of Science:

  • Neuroscience
  • Neurology
  • Genetics

Background:

  • Effective treatment for neurodegenerative diseases like amyotrophic lateral sclerosis (ALS) depends on early therapeutic intervention.
  • Advances in gene therapies, including antisense oligonucleotides and viral vectors, necessitate a deeper understanding of pre-symptomatic disease stages.
  • Studying pre-symptomatic ALS requires a clear conceptual framework to define and analyze this early phase.

Purpose of the Study:

  • To propose a novel conceptual framework for understanding pre-symptomatic amyotrophic lateral sclerosis (ALS).
  • To distinguish ALS as a clinical syndrome from ALS as a disease entity.
  • To define two distinct stages of pre-symptomatic ALS: pre-manifest and prodromal.

Main Methods:

  • Literature review of neurodegenerative diseases and personal clinical experience.
  • Development of a conceptual model to characterize pre-symptomatic ALS.
  • Articulation of the importance of biomarker development and definition of phenoconversion.

Main Results:

  • A proposed model distinguishes ALS syndrome from ALS disease.
  • Pre-symptomatic ALS is characterized into two stages: pre-manifest and prodromal.
  • The critical role of biomarker development and an operational definition of phenoconversion are presented.

Conclusions:

  • The proposed framework aims to accelerate research into pre-symptomatic ALS.
  • This model is expected to facilitate the design and implementation of early intervention and disease prevention trials for ALS.
  • A better understanding of pre-symptomatic ALS is crucial for developing effective therapeutic strategies.