Presented at the 27th International Symposium on Amyotrophic Lateral Sclerosis and Motor Neurone Disease, Dublin Ireland, December 2016.

Objectives:

  1. To develop a meaningful operational definition of “ALS resistance” which captures patients with unexpectedly long plateaus or large and sustained reversals
  2. To determine whether ALS resistance is regional (occurring across multiple ALSFRS-R questions related to the same body part) or random (and thus more likely to be due to noise).
  3. To look for differences in demographics, medications and laboratory values between resistant patients and those with more typical progression.

Conclusions: We compared resistant and more typically progressive patients in 40 demographic categories, 333 medications, 114 adverse events and 111 labs using t-tests. After adjusting for multiple comparisons, we found no significant differences. There may be a genetic component, as seen in HIV elite controllers. Our definition of ALS resistance (2 ALSFRS-R points at least 6 months apart that are 2 standard deviations better than predicted) appears clinically meaningful and should allow prospective identification of a reasonably large cohort of resistant patients for further studies.

Authors: Richard S. Bedlack, David Ennist, Albert Taylor, Mike Keymer

Final-P197-Duke-Origent-Resistance-2016-ALS-MND-Symp

 

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