Susanna Summa1, Tommaso Schirinzi2, Giuseppe Massimo Bernava3, Alberto Romano4, Martina Favetta5, Enza Maria Valente6, Enrico Bertini7, Enrico Castelli8, Maurizio Petrarca9, Giovanni Pioggia10, Gessica Vasco11. 1. MARlab, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy. Electronic address: susanna.summa@opbg.net. 2. MARlab, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy; Department of Systems Medicine, University of Roma Tor Vergata, Rome, Italy. Electronic address: tommaso.schirinzi@opbg.net. 3. Institute for Biomedical Research and Innovation (IRIB-CNR), Via Torre Bianca, Mortelle, Istituto Marino, 98164 Messina, Italy. Electronic address: m.bernava@irib.cnr.it. 4. MARlab, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy. Electronic address: alberto.romano@opbg.net. 5. MARlab, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy. Electronic address: martina.favetta@opbg.net. 6. Department of Molecular Medicine, Unit of Genetics, Università degli studi di Pavia, Pavia, Italy; IRCCS Mondino Foundation, Pavia, Italy. Electronic address: enzamaria.valente@unipv.it. 7. Unit of Neuromuscolar and Neurodegenerative Diseases, Department of Neurosciences, IRCCS Bambino Gesù Children's Hospital, Rome, Italy. Electronic address: enricosilvio.bertini@opbg.net. 8. MARlab, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy. Electronic address: enrico.castelli@opbg.net. 9. MARlab, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy. Electronic address: maurizio.petrarca@opbg.net. 10. Institute for Biomedical Research and Innovation (IRIB-CNR), Via Torre Bianca, Mortelle, Istituto Marino, 98164 Messina, Italy. Electronic address: g.pioggia@irib.cnr.it. 11. MARlab, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy. Electronic address: gessica.vasco@opbg.net.
Abstract
BACKGROUND AND OBJECTIVE: Early onset ataxias (EOAs) are a heterogeneous group of neurological conditions, responsible for severe motor disability in paediatric age, which still lack reliable outcome measures. Available scales to assess ataxia, such as the Scale for Assessment and Rating of Ataxia (SARA), are based on subjective assessment of specific motor and language tasks by an examiner, and therefore is age dependent and lacks accuracy in detecting small variations in disease severity. In last years, novel technologies, including computer interfaces and videogames, have emerged for clinical applications and the advent of Internet of Medical Things and of Information Communication Technology have allowed the remote control of such technologies. This pilot study describes a newly developed tool (SaraHome) for the assessment at home of EOA evaluating its feasibility and acceptability on a small sample of children. METHODS: Ten EOA children and ten caregivers have been enrolled for a preliminary outpatient evaluation. The Microsoft Kinect 2.0 and Leap Motion Controller (LMC) connected to a personal computer with an ad hoc software have been set-up, for the acquisition of standardized motor tasks performed by the patients with the caregivers' assistance. Acceptance and practicability have been tested by QUEST 2.0 and IMI questionnaires in caregivers and patients respectively. RESULTS: The SaraHome software was developed, based on a collection of services provided by a complex architecture that consists of a Restful interface, which enables to access a series of plugins for the execution of different tasks. A graphical user interface allows the acquisition of the patient movements while performing a motor task. A protocol of standard tasks inspired by SARA was established, and a system of video-assisted instruction provided. The set-up for the optimal acquisition of such protocol by Kinect and LMC has been defined. Both patients and caregivers accomplished the SaraHome assessment with good feedback at the technology acceptance questionnaires. CONCLUSIONS: SaraHome represents a newly developed tool for the assessment of ataxia in patients, resulting from the integration of low-cost and easy-accessible technologies. This pilot application highlighted the feasibility and the acceptability of the system, suggesting the potential use in clinical practice.
BACKGROUND AND OBJECTIVE: Early onset ataxias (EOAs) are a heterogeneous group of neurological conditions, responsible for severe motor disability in paediatric age, which still lack reliable outcome measures. Available scales to assess ataxia, such as the Scale for Assessment and Rating of Ataxia (SARA), are based on subjective assessment of specific motor and language tasks by an examiner, and therefore is age dependent and lacks accuracy in detecting small variations in disease severity. In last years, novel technologies, including computer interfaces and videogames, have emerged for clinical applications and the advent of Internet of Medical Things and of Information Communication Technology have allowed the remote control of such technologies. This pilot study describes a newly developed tool (SaraHome) for the assessment at home of EOA evaluating its feasibility and acceptability on a small sample of children. METHODS: Ten EOA children and ten caregivers have been enrolled for a preliminary outpatient evaluation. The Microsoft Kinect 2.0 and Leap Motion Controller (LMC) connected to a personal computer with an ad hoc software have been set-up, for the acquisition of standardized motor tasks performed by the patients with the caregivers' assistance. Acceptance and practicability have been tested by QUEST 2.0 and IMI questionnaires in caregivers and patients respectively. RESULTS: The SaraHome software was developed, based on a collection of services provided by a complex architecture that consists of a Restful interface, which enables to access a series of plugins for the execution of different tasks. A graphical user interface allows the acquisition of the patient movements while performing a motor task. A protocol of standard tasks inspired by SARA was established, and a system of video-assisted instruction provided. The set-up for the optimal acquisition of such protocol by Kinect and LMC has been defined. Both patients and caregivers accomplished the SaraHome assessment with good feedback at the technology acceptance questionnaires. CONCLUSIONS: SaraHome represents a newly developed tool for the assessment of ataxia in patients, resulting from the integration of low-cost and easy-accessible technologies. This pilot application highlighted the feasibility and the acceptability of the system, suggesting the potential use in clinical practice.
Authors: Pierre Cabaraux; Sunil K Agrawal; Huaying Cai; Rocco Salvatore Calabro; Carlo Casali; Loic Damm; Sarah Doss; Christophe Habas; Anja K E Horn; Winfried Ilg; Elan D Louis; Hiroshi Mitoma; Vito Monaco; Maria Petracca; Alberto Ranavolo; Ashwini K Rao; Serena Ruggieri; Tommaso Schirinzi; Mariano Serrao; Susanna Summa; Michael Strupp; Olivia Surgent; Matthis Synofzik; Shuai Tao; Hiroo Terasi; Diego Torres-Russotto; Brittany Travers; Jaimie A Roper; Mario Manto Journal: Cerebellum Date: 2022-04-12 Impact factor: 3.847
Authors: Ha Tran; Khoa D Nguyen; Pubudu N Pathirana; Malcolm K Horne; Laura Power; David J Szmulewicz Journal: J Neuroeng Rehabil Date: 2020-12-04 Impact factor: 4.262