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Related Experiment Video

Updated: May 7, 2026

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

Published on: July 16, 2021

Amyotrophic lateral sclerosis disease progression model.

Roberto Gomeni¹, Maurizio Fava,

¹PharmacoMetrica , La Fouillade , France.

Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration

|September 28, 2013

Summary

This summary is machine-generated.

Researchers developed a model to track amyotrophic lateral sclerosis (ALS) progression. The study identified distinct slow and fast disease progression clusters in patients, highlighting treatment heterogeneity.

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ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

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ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

Published on: July 29, 2007

Area of Science:

Neurology
Biostatistics

Background:

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease.
Understanding ALS disease progression is crucial for clinical trial design and patient management.

Purpose of the Study:

To develop a longitudinal model for amyotrophic lateral sclerosis (ALS) disease progression using the ALSFRS-R.
To create a probabilistic model identifying distinct ALS progression trajectories over 12 months.

Main Methods:

Utilized the PRO-ACT database including 338 patients on placebo.
Applied a non-linear Weibull model for disease progression and stepwise logistic regression.
Employed ROC curve analysis to determine classification cut-offs for progression rates.

Main Results:

Identified two distinct clusters of ALS disease progression: slow (46%) and fast (54%).
Slow progressors showed a 13% change from baseline, while fast progressors exhibited a 49% change.
ALSFRS-R measurements at 2-4 weeks could classify patients into slow or fast progression groups.

Conclusions:

ALS disease progression is highly heterogeneous, as evidenced by ALSFRS-R changes.
Disease progression models can aid in patient enrichment strategies for clinical trials.
Controlling heterogeneity in trial populations is essential for accurate therapeutic evaluation.