Myotonic dystrophy health index: Correlations with clinical tests and patient function.

ABSTRACT:

Introduction

The Myotonic Dystrophy Health Index (MDHI) is a disease-specific patient-reported outcome measure. Here, we examine the associations between the MDHI and other measures of disease burden in a cohort of individuals with myotonic dystrophy type-1 (DM1).

Methods

We conducted a cross-sectional study of 70 patients with DM1. We examined the associations between MDHI total and subscale scores and scores from other clinical tests. Participants completed assessments of strength, myotonia, motor and respiratory function, ambulation, and body composition. Participants also provided blood samples, underwent physician evaluations, and completed other patient-reported outcome measures.

Results

MDHI total and subscale scores were strongly associated with muscle strengt

SUBMITTER: Heatwole C 

PROVIDER: S-EPMC4979973 | biostudies-literature | 2016 Feb

REPOSITORIES: biostudies-literature