Dataset Information

2-Year Change in Revised Hammersmith Scale Scores in a Large Cohort of Untreated Paediatric Type 2 and 3 SMA Participants.

ABSTRACT: The Revised Hammersmith Scale (RHS) is a 36-item ordinal scale developed using clinical expertise and sound psychometrics to investigate motor function in participants with Spinal Muscular Atrophy (SMA). In this study, we investigate median change in the RHS score up to two years in paediatric SMA 2 and 3 participants and contextualise it to the Hammersmith Functional Motor Scale-Expanded (HFMSE). These change scores were considered by SMA type, motor function, and baseline RHS score. We consider a new transitional group, spanning crawlers, standers, and walkers-with-assistance, and analyse that alongside non-sitters, sitters, and walkers. The transitional group exhibit the most definitive change score trend, with an average 1-year decline of 3 points. In the weakest patients, we are most able to detect positive change in the RHS in the under-5 age group, whereas in the stronger patients, we are most able to detect decline in the RHS in the 8-13 age group. The RHS has a reduced floor effect compared to the HFMSE, although we show that the RHS should be used in conjunction with the RULM for participants scoring less than 20 points on the RHS. The timed items in the RHS have high between-participant variability, so participants with the same RHS total can be differentiated by their timed test items.

SUBMITTER: Stimpson G

PROVIDER: S-EPMC10004034 | biostudies-literature | 2023 Feb

REPOSITORIES: biostudies-literature

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2-Year Change in Revised Hammersmith Scale Scores in a Large Cohort of Untreated Paediatric Type 2 and 3 SMA Participants.

Stimpson Georgia G Ramsey Danielle D Wolfe Amy A Mayhew Anna A Scoto Mariacristina M Baranello Giovanni G Muni Lofra Robert R Main Marion M Milev Evelin E Coratti Giorgia G Pane Marika M Sansone Valeria V D'Amico Adele A Bertini Enrico E Messina Sonia S Bruno Claudio C Albamonte Emilio E Mazzone Elena Stacy ES Montes Jacqueline J Glanzman Allan M AM Zolkipli-Cunningham Zarazuela Z Pasternak Amy A Duong Tina T Dunaway Young Sally S Civitello Matthew M Marini-Bettolo Chiara C Day John W JW Darras Basil T BT De Vivo Darryl C DC Finkel Richard S RS Mercuri Eugenio E Muntoni Francesco F

Journal of clinical medicine 20230228 5

The Revised Hammersmith Scale (RHS) is a 36-item ordinal scale developed using clinical expertise and sound psychometrics to investigate motor function in participants with Spinal Muscular Atrophy (SMA). In this study, we investigate median change in the RHS score up to two years in paediatric SMA 2 and 3 participants and contextualise it to the Hammersmith Functional Motor Scale-Expanded (HFMSE). These change scores were considered by SMA type, motor function, and baseline RHS score. We conside ...[more]

PMID: 36902710

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Project description:BackgroundClimate change anxiety, that is worry and fear in relation to the awareness of the impacts of climate change, is widely observed around the world. Some evidence suggests that while climate change anxiety can, at times, be adaptive, a growing body of research has reported that climate change anxiety is also related to a range of negative mental health outcomes and psychological distress. Currently, however, there is limited ability to assess for elevated levels of climate change anxiety and to identify those who may need support. The present study, therefore, aimed to develop clinical cut-off scores on a measure of climate change anxiety.MethodsA largely representative sample of Australian young adults (aged 16-25 years) completed measures of psychological distress (Depression, Anxiety and Stress Scale-21) and the Climate Change Anxiety Scale. Markers of clinically meaningful psychological distress - elevated depression, anxiety, and stress symptoms-were used to classify cases. Receiver Operating Characteristics analyses were performed to assess the predictive ability of the indicators of psychological distress (mild, moderate, severe, and extremely severe thresholds of anxiety, depression, and stress symptoms) for detecting climate change anxiety and to ascertain optimal cut-off scores.ResultsThe Area Under the Curve was acceptable to moderate for detecting climate change anxiety across all analyses. Across symptom severity thresholds and markers of psychological distress, based on consideration of balancing sensitivity and specificity, results consistently suggested that a cut-score of 21 was indicative of mild-moderate climate change anxiety, with a cut-off score of 23 indicating severe-extremely severe climate change anxiety.ConclusionsThe proposed cut-offs can feasibly be used to identify those with elevated climate change anxiety. Use of these cut-off scores can inform research as well as be used to guide screening, assessment, and inform clinical practice. Results also highlight a high rate of climate change anxiety in young adults.

Dataset Information

2-Year Change in Revised Hammersmith Scale Scores in a Large Cohort of Untreated Paediatric Type 2 and 3 SMA Participants.

Publications

2-Year Change in Revised Hammersmith Scale Scores in a Large Cohort of Untreated Paediatric Type 2 and 3 SMA Participants.

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