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ACTIVE (Ability Captured Through Interactive Video Evaluation) workspace volume video game to quantify meaningful change in spinal muscular atrophy
Author(s) -
Alfano Lindsay N,
Miller Natalie F,
Iammarino Megan A,
Moore Clingenpeel Melissa,
Lowes Suzanne L,
Dugan Margaret E,
Kissel John T,
Al Zaidy Samiah,
Tsao ChangYong,
Lowes Linda P
Publication year - 2020
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.14230
Subject(s) - spinal muscular atrophy , workspace , video game , computer science , physical medicine and rehabilitation , video recording , volume (thermodynamics) , medicine , human–computer interaction , multimedia , artificial intelligence , algorithm , sma* , physics , quantum mechanics , robot
Aim To evaluate the utility of Ability Captured Through Interactive Video Evaluation ( ACTIVE ) scaled scores to quantify meaningful change in individuals with spinal muscular atrophy ( SMA ) types 2 or 3 due to disease progression or treatment. Method ACTIVE is a custom‐designed video game that measures workspace volume ( WSV ). Participants included 62 individuals with SMA (mean age [ SD ] 10y 9mo [5y], range 2y 9mo–24y) and 362 frequency‐matched controls (mean age [ SD ] 10y 9mo [3y 6mo], range 3y 2mo–24y 9mo). Participants completed ACTIVE , other traditional assessments, and patient‐reported outcomes. Responsiveness to change was evaluated by comparing longitudinal data on untreated participants to those receiving Spinraza. Results ACTIVE was significantly correlated to the Hammersmith Functional Motor Scales Expanded and Revised Upper Limb Module ( ρ =0.85 and ρ =0.92 respectively; p <0.001). Relevance to patients and families was established by strong correlations to the Patient Reported Outcomes Measurement Information System self‐ and parent proxy‐measures of upper extremity ability ( ρ =0.63 and ρ =0.70 respectively; p <0.001). Responsiveness to change was demonstrated by significant change in scaled scores after treatment (median 15.9 points, Wilcoxon signed‐rank test p <0.01). A preliminary minimum clinically important difference is presented. Interpretation These results suggest that ACTIVE WSV scores are a meaningful assessment with which to quantify change over time in individuals with SMA types 2 and 3. What this paper adds Ability Captured Through Interactive Video Evaluation (ACTIVE) quantifies upper extremity function in spinal muscular atrophy. ACTIVE's scaled workspace volume strongly correlates to self‐ and parent‐report of function. ACTIVE quantifies meaningful change after treatment.