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A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS
Published on: October 6, 2015
A proposed staging system for amyotrophic lateral sclerosis.
Jose C Roche1, Ricardo Rojas-Garcia, Kirsten M Scott
1MRC Centre for Neurodegeneration Research, King's College London, Institute of Psychiatry, London SE5 8AF, UK.
Brain : a Journal of Neurology
|January 25, 2012
Summary
Researchers developed a new staging system for amyotrophic lateral sclerosis (ALS), a neurodegenerative disease. This system uses clinical milestones to objectively measure disease progression, aiding patient care and clinical trials.
Area of Science:
- Neurology
- Neurodegenerative Diseases
- Clinical Research
Background:
- Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with a median survival of 2-3 years.
- Existing classification and prognostic models for ALS lack a standardized staging system.
- A universal staging system for ALS is needed for improved patient care, resource allocation, research, and clinical trial design.
Purpose of the Study:
- To define easily identifiable clinical milestones in the disease course of ALS.
- To establish objective criteria for staging ALS progression.
- To provide a universal measure of disease progression impacting prognosis and treatment.
Main Methods:
- Analysis of a clinical database of 1471 patients with ALS from 1993-2007.
- Definition of milestones: symptom onset, diagnosis, involvement of second/third regions, gastrostomy, and non-invasive ventilation.
- Standardization of milestone timings as proportions of disease duration.
Main Results:
- Milestones occurred at predictable proportions of the disease course.
- Diagnosis at 35%, second region involvement at 38%, third region at 61%, gastrostomy at 77%, and non-invasive ventilation at 80% of disease duration.
- A proposed 4-stage staging system based on these milestones.
Conclusions:
- A simple, objective staging system for ALS based on clinical milestones is proposed.
- This staging system can aid in patient care, resource allocation, and clinical trial design.
- Further validation in diverse populations and databases is required.
