Background: The Dyskinesia Impairment Scale (DIS) is a new assessment scale for dystonia and choreoathetosis in children and youth with dyskinetic cerebral palsy. Today, the Burke-Fahn-Marsden Dystonia Rating Scale (BFM) is mostly used to assess dystonia in children with inherited dystonia. The aim of this study was to assess reliability and validity of the DIS in children and youth with inherited or idiopathic dystonia.
Methods: Reliability was measured by (1) the intraclass correlation coefficients (ICCs) for inter-rater and test-retest reliability, as well as (2) standard error of measurement (SEM) and minimal detectable difference (MDD). For concurrent validity of the DIS-dystonia subscale, the BFM was administered.
Results: In total, 11 males and 9 females (median age 16 years and 7 months, range 6 to 24 years) were included. For inter-rater reliability, the ICCs for the DIS total score and the dystonia and choreoathetosis subscale scores were 0.83, 0.87, and 0.71, respectively. For test-retest reliability, the ICCs for the DIS total score and the dystonia and choreoathetosis subscale scores were 0.95, 0.88, and 0.93, respectively. The SEM and MDD for the total DIS were 3.98% and 11.04%, respectively. The Spearman correlation coefficient between the dystonia subscale and the BFM was 0.88 ( < 0.01).
Conclusions: Good to excellent inter-rater, test-retest reliability, and validity were found for the total DIS and the dystonia subscale. The choreoathetosis subscale showed moderate inter-rater reliability and excellent test-retest reliability. The DIS may be a promising tool to assess dystonia and choreoathetosis in children and young adults with inherited or idiopathic dystonia.
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http://dx.doi.org/10.3390/jcm9082597 | DOI Listing |
Cureus
September 2024
General Medicine, Sri Ramaswamy Memorial (SRM) Medical College Hospital and Research Centre, SRM Institute of Science and Technology (SRMIST), Chengalpattu, IND.
Mov Disord
September 2024
Edmond J. Safra Program in Parkinson's Disease, Rossy PSP Centre and the Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, Toronto, Ontario, Canada.
Acute presentation of severe motor disorders is a diagnostic and management challenge. We define severe acute motor exacerbations (SAME) as acute/subacute motor symptoms that persist for hours-to-days with a severity that compromise vital signs (temperature, breath, and heart rate) and bulbar function (swallowing/dysphagia). Phenomenology includes dystonia, choreoathetosis, combined movement disorders, weakness, and hemiplegic attacks.
View Article and Find Full Text PDFJ Biomech
August 2024
Department of Rehabilitation Sciences, KU Leuven Bruges, Bruges, Belgium; Department of Rehabilitation Sciences, KU Leuven, Leuven, Belgium; Department of Rehabilitation Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, the Netherlands; Rehabilitation & Development, Amsterdam Movement Sciences, Amsterdam, the Netherlands. Electronic address:
This study aimed to evaluate clinical utility of 2D-markerless motion analysis (2DMMA) from a single camera during a reaching-sideways-task in individuals with dyskinetic cerebral palsy (DCP) by determining (1) concurrent validity by correlating 2DMMA against marker-based 3D-motion analysis (3DMA) and (2) construct validity by assessing differences in 2DMMA features between DCP and typically developing (TD) peers. 2DMMA key points were tracked from frontal videos of a single camera by DeepLabCut and accuracy was assessed against human labelling. Shoulder, elbow and wrist angles were calculated from 2DMMA and 3DMA (as gold standard) and correlated to assess concurrent validity.
View Article and Find Full Text PDFJ Autism Dev Disord
July 2024
Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of medical Education and Research (PGIMER), Chandigarh, 160012, India.
Background: The data on specific comorbidities in children with dyskinetic cerebral palsy (DCP) is limited. We evaluated the pattern of comorbidities and health related quality of life (HRQOL) in these children and compared them between etiological and motor impairment subgroups.
Methodology: This cross-sectional study was conducted over 18 months in children with DCP of both sex, and age between one and 14 years.
Pediatr Neurol
September 2024
Pediatric Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India. Electronic address:
Background: Trihexyphenidyl and clonazepam are commonly used to treat dystonia in children with cerebral palsy (CP). However, there is a notable gap in the literature when it comes to studies that combine these first-line agents for the management of dystonia.
Methods: This open-label, randomized controlled trial aimed to compare the efficacy of adding oral clonazepam to trihexyphenidyl (THP + CLZ) versus using trihexyphenidyl alone (THP) in reducing the severity of dystonia, as measured by the Barry-Albright Dystonia (BAD) score.
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