P.54 Assessment of fine motor abilities using new touchscreen application, among children with spinal muscular atrophy (SMA)- a pilot study

Abstract

Spinal muscular atrophy (SMA) is a genetic neurodegenerative disease characterized by severe muscle weakness and atrophy. Advances in novel drug therapies has changed dramatically the natural history of the disease and outcome measures are used to assess the clinical response to therapy. Recent research has given rise to a central issue regarding the low sensitivity and limited ability of standard SMA assessment tools in detecting the subtle changes in fine motor abilities, which occur following the use of those therapies. Furthermore, there is lack of standardized and ecological tools for assessment of fine motor abilities and changes in SMA patients' daily functions. The purpose of this study was to evaluate the use touchscreen app. as an assessment tool for detecting changes in the upper extremity (UE) motor functions among SMA patients as a response to the use of the genetic therapies. 14 patients aged 6- 23 years were recruited. Nine patients were diagnosed with SMA type 2 and five were diagnosed with SMA type 3. The patients were evaluated every six months. The study included assessment of fine motor functions, using the touchscreen app which included temporal (e.g. reaction times, duration) and accuracy (touch all corners, double tapping, drag items and pinch ability) tasks. Our preliminary tests results show that the App. is able to detect fine changes in the performance, mainly in the parameters of reaction time and duration in most of the tasks. The participants were highly motivated to use the App. comparing to standard tools and it was easy to use among all ages and types of SMA. The results of this pilot study may contribute to the new developing knowledge about the influence of the genetic therapies on motor functions of the UE in the SMA population. In addition, the results may help to establish assessments for SMA that are ecological and more sensitive in detecting changes in motor abilities and function in daily activities.