Defining Hand Stereotypies in Rett Syndrome: A Movement Disorders Perspective.

ABSTRACT:

Introduction

Hand stereotypies (HS) are a primary diagnostic criterion for Rett syndrome (RTT) but are difficult to characterize and quantify systematically.

Methods

We collected video on 27 girls (2-12 years of age) with classic RTT who participated in a mecasermin trial. The present study focused exclusively on video analyses, by reviewing two five-minute windows per subject to identify the two most common HS. Three raters with expertise in movement disorders independently rated the five-minute windows using standardized terminology to determine the level of agreement. We iteratively refined the protocol in three stages to improve descriptive accuracy, categorizing HS as "central" or "peripheral," "simple" or "complex," scoring each hand separately. Inter-rater agreement w

SUBMITTER: Dy ME 

PROVIDER: S-EPMC5624791 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature