Abstract Details

Title Development of a Clinical Staging System for Amyotrophic Lateral Sclerosis

Topic Anterior Horn

Presentation(s) P07 - (-)

Poster/Presentation
Number 071

Objective

Background BACKGROUND: Clinical trials track treatment effect on function in ALS patients using functional rating scales, e.g. the ALS Functional Rating Scale - Revised (ALSFRS-R). It may be appropriate to evaluate ALS patients' natural disease course using defined clinical stages.

Design/Methods DESIGN/METHODS: Critical milestones in ALS progression were defined by ALSFRS item score changes in 4 key domains: swallowing, walking/self-care, communicating, and breathing. Stages were defined as: 0 - functional involvement but no loss of independence on any domain; 1-4 -number of domains in which independence was lost; 5 - death. A prospective analysis of consecutively enrolled patients (?18 to ?80 yrs) from 11 Italian ALS centers was used to validate these defined stages. Patients within the catchment area with definite, probable or probable laboratory supported ALS, without serious comorbidities were included. At study initiation and at 4, 8, and 12 months, patients' function (10 item ALSFRS), quality of life (QoL; Short Form-36[SF-36]) and direct costs were evaluated.

Results RESULTS: Of 130 patients included, at baseline 48.7% patients were at Stage 0, 27.7% Stage 1, 11.8% Stage 2, 3.4% Stage 3, and 8.4% Stage 4. At 12 months, 15.7% patients were at Stage 0, 21.7% Stage 1, 15.7% Stage 2, 10.4% Stage 3, 13.9% Stage 4, and 22.6% Stage 5. Mean (±SE) overall ALSFRS score was 31.0 (±0.4), 22.7 (±0.4), 15.8 (±0.5), 9.5 (±0.7), 3.2 (±0.5) in Stages 0-4, respectively. SF-36 physical and mental health composite scores decreased progressively with each stage. Direct costs increased with increasing stages.

Conclusions CONCLUSIONS: The proposed staging system correlated well with functional, QoL and direct cost assessments.

Authors/Disclosures
Adriano Chio, MD, FAAN (Dept. of Neuroscience, University of Turin) PRESENTER	Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cytokinetics. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Corcept.
	No disclosure on file
Gabriele Mora, MD	Dr. Mora has nothing to disclose.
	No disclosure on file
Graziella Filippini, MD	No disclosure on file
	No disclosure on file

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