Abstract
There are no currently available disease-modifying pharmacological treatments for most of the chronic hereditary ataxias; thus, effective rehabilitative strategies are crucial to help improve symptoms and therefore the quality of life. We propose to gather all available evidence on the use of video games, exergames, and apps for tablet and smartphone for the rehabilitation, diagnosis, and assessment of people with ataxias. Relevant literature published up to June 8, 2020, was retrieved searching the databases PubMed, ISI Web of Science, and the Cochrane Database. Data were extracted using a standardized form, and their methodological quality was assessed using RoB and QUADAS-2. Six studies of 434 retrieved articles met the predefined inclusion/exclusion criteria. Two of them were diagnostic, while 4 were experimental studies. Studies included participants ranging from 9 to 28 in trials and 70 to 248 in diagnostic studies. Although we found a small number of trials and of low methodological quality, all of them reported an improvement of motor outcomes and quality of life as measured by specific scales, including the SARA, BBS, DHI, and SF-36 scores. The main reason for such low quality in trials was that most of them were small and uncontrolled, thus non-randomized and unblinded. As video games, exergames, serious games, and apps were proven to be safe, feasible, and at least as effective as traditional rehabilitation, further and more high-quality studies should be carried out on the use of these promising technologies in people with different types of ataxia.
Highlights
Ataxia, or lack of voluntary coordination of muscle movements, has an overall prevalence of around 26 per 100,000 in European pediatric population [1], 8.4 per 100,000 for idiopathic late-onset cerebellar ataxia (LOCA), and 1.8 per 100,000 for inheritedLOCA [2]
Ataxia is a common neurological sign that might be due to several different neurological conditions, including brain tumors, brain injuries, stroke, infections, toxicity, or genetic causes
This systematic review was carried out according to the methodology reported in the Cochrane Handbook for Systematic Reviews [20] and followed the PRISMA statement for reporting systematic reviews and meta-analyses [21, 22]
Summary
Lack of voluntary coordination of muscle movements, has an overall prevalence of around 26 per 100,000 in European pediatric population [1], 8.4 per 100,000 (95% CI 7.2 to 11.6) for idiopathic late-onset cerebellar ataxia (LOCA), and 1.8 per 100,000 (95% CI 0.8 to 2.7) for inheritedLOCA [2]. Lack of voluntary coordination of muscle movements, has an overall prevalence of around 26 per 100,000 in European pediatric population [1], 8.4 per 100,000 (95% CI 7.2 to 11.6) for idiopathic late-onset cerebellar ataxia (LOCA), and 1.8 per 100,000 (95% CI 0.8 to 2.7) for inherited. Based on the population living in Europe, we can estimate about 525,000 cases in all ages (1300 in pediatric population and 431,200 and 92,500 for idiopathic and inherited LOCA, respectively) (https://ec.europa.eu/eurostat). Ataxia is a common neurological sign that might be due to several different neurological conditions, including brain tumors, brain injuries, stroke, infections (e.g., varicella), toxicity, or genetic causes. The diagnosis of the underlying cause is a crucial step, and might be a long process in case of chronic or episodic ataxias, due to the rarity and complexity of these conditions [3]. Ataxia is part of a multisystem dysfunction, which may cause intellectual disability and/or other neurological symptoms, spine deformities such as Cerebellum (2021) 20:361–373 scoliosis, and/or several other disorders affecting different organs (e.g., cardiomyopathy, congenital cataracts, optic nerve atrophy, retinal dystrophy, and deafness) [4,5,6,7,8]
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