Defining Hand Stereotypies in Rett Syndrome: A Movement Disorders Perspective

Title

Defining Hand Stereotypies in Rett Syndrome: A Movement Disorders Perspective

Creator

Dy M E; Waugh J L; Sharma N; O'Leary H; Kapur K; D'Gama A M; Sahin M; Urion D K; Kaufmann W E

Publisher

Pediatric Neurology

Date

2017

Subject

Pediatrics; definitions; Neurosciences & Neurology; Rett syndrome; girls; autism; actigraphy; hand stereotypies; operational; regression; video analysis; tone and motor problems; tool development; scale development; hand stereotypies

Description

INTRODUCTION: Hand stereotypies (HS) are a primary diagnostic criterion for Rett syndrome (WIT) 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 was analyzed using Kappa statistics. RESULTS: In the initial protocol evaluating HS by video, inter-rater agreement was 20.7%. In the final protocol, inter-rater agreement for the two most frequent HS was higher than the initial protocol at 50%. CONCLUSION: Phenotypic variability makes standardized evaluation of HS in RTT a challenge; we achieved only 50% level of agreement and only for the most frequent HS. Therefore, objective measures are needed to evaluate HS.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

Dy M E; Waugh J L; Sharma N; O'Leary H; Kapur K; D'Gama A M; Sahin M; Urion D K; Kaufmann W E, “Defining Hand Stereotypies in Rett Syndrome: A Movement Disorders Perspective,” Pediatric Palliative Care Library, accessed September 22, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16641.

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