Efficient design and analysis of randomized controlled trials in rare neurological diseases: An example in Guillain-Barré syndrome

PLoS One. 2019 Feb 20;14(2):e0211404. doi: 10.1371/journal.pone.0211404. eCollection 2019.

Abstract

Background: Randomized controlled trials (RCTs) pose specific challenges in rare and heterogeneous neurological diseases due to the small numbers of patients and heterogeneity in disease course. Two analytical approaches have been proposed to optimally handle these issues in RCTs: covariate adjustment and ordinal analysis. We investigated the potential gain in efficiency of these approaches in rare and heterogeneous neurological diseases, using Guillain-Barré syndrome (GBS) as an example.

Methods: We analyzed two published GBS trials with primary outcome 'at least one grade improvement' on the GBS disability scale. We estimated the treatment effect using logistic regression models with and without adjustment for prognostic factors. The difference between the unadjusted and adjusted estimates was disentangled in imbalance (random differences in baseline covariates between treatment arms) and stratification (change of the estimate due to covariate adjustment). Second, we applied proportional odds regression, which exploits the ordinal nature of the GBS disability score. The standard error of the estimated treatment effect indicated the statistical efficiency.

Results: Both trials were slightly imbalanced with respect to baseline characteristics, which was corrected in the adjusted analysis. Covariate adjustment increased the estimated treatment effect in the two trials by 8% and 18% respectively. Proportional odds analysis resulted in lower standard errors indicating more statistical power.

Conclusion: Covariate adjustment and proportional odds analysis most efficiently use the available data and ensure balance between the treatment arms to obtain reliable and valid treatment effect estimates. These approaches merit application in future trials in rare and heterogeneous neurological diseases like GBS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Guillain-Barre Syndrome / therapy*
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Logistic Models
  • Methylprednisolone / therapeutic use
  • Nervous System Diseases / therapy*
  • Odds Ratio
  • Plasma Exchange
  • Randomized Controlled Trials as Topic / methods
  • Randomized Controlled Trials as Topic / statistics & numerical data
  • Rare Diseases / therapy*

Substances

  • Immunoglobulins, Intravenous
  • Methylprednisolone

Grants and funding

BJ received unrestricted research support from the Netherlands Organization for Health Research and Development, Erasmus MC, Prinses Beatrix Spierfonds, GBS-CIDP Foundation International, Baxalta, CSLBehring, Grifols, and Annexon. PvD received unrestricted research support from the Prinses Beatrix Spierfonds, Janivo Stichting, Baxalta, Grifols, and Sanquin Plasma Pharmaceuticals. No other disclosures were reported.