Abstract Details

Title Prognostic Factors Affecting ALS Progression through Disease Tollgates

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 378

Objective Understanding factors affecting variability in amyotrophic lateral sclerosis (ALS) progression.

Background Individualized models displaying progression of ALS through clinically meaningful critical events, (tollgates), is lacking.

Design/Methods

We assessed timing of several critical events, i.e., tollgates, in ALS progression. We augmented 6,366 patients’ data from the PRO-ACT database with tollgate-passed information using binary classification. We then derived time trajectories of tollgate passage using Kaplan-Meier analyses. We used log-rank test to identify significant prognostic factors for ALS progression pathways, and decision-tree-based classification to specify ALS phenotypes (indicators for early involvement of body segments) displaying different paces of disease progression through ALS tollgates.

Results Standard prognostic factors (e.g., gender) and factors related to ALS history significantly affected timing of tollgate passage. For example, by the third year after the first visit, 80-100% of bulbar-onset patients vs. 40-50% of limb-onset patients lost the ability to talk and started using a feeding tube. Similarly, around 70% of females vs. 50% of males passed these tollgates by year three. Particular standard factors affected only late ALS tollgates, i.e., advanced bulbar tollgates occurred earlier among females; while gender did not affect early/intermediate limb tollgates. The impairment level at the first visit impacted subsequent ALS progression in the considered segment, while the affected segments the first visit further characterized ALS progression speed. For example, patients with normal speech (Tollgate Level 0) at the first visit had less than a 10% likelihood of losing speech within a year. For patients with Tollgate Level 1 (affected speech), this probability increased to 23% with early swallowing and leg involvement, and to 50% with swallowing impairment and normal leg function.

Conclusions

Tollgate-related factors had more pronounced effect on ALS progression through tollgates compared to standard factors. Future research may jointly consider all risk factors for characterizing groups with different progression aggressiveness and individualize ALS care.

Authors/Disclosures
Brian Crum, MD (Mayo Clinic) PRESENTER	Dr. Crum has nothing to disclose.
	No disclosure on file
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