Abstract
BackgroundFriedreich ataxia (FRDA) is a disease with neurological and systemic involvement. Clinical assessment tools commonly used for FRDA become less effective in evaluating decay in patients with advanced FRDA, particularly when they are in a wheelchair. Further motor worsening mainly impairs upper limb function. In this study, we tested if serious games (SG) developed for rehabilitation can be used as an assessment tool for upper limb function even in patients with advanced FRDA.MethodsA specific SG has been developed for physical rehabilitation of patients suffering from neurologic diseases. The use of this SG, coupled with Kinect sensor, has been validated to perform functional evaluation of the upper limbs with healthy subjects across lifespan. Twenty-seven FRDA patients were included in the study. Patients were invited to perform upper limb rehabilitation exercises embedded in SG. Motions were recorded by the Kinect and clinically relevant parameters were extracted from the collected motions. We tested if the existence of correlations between the scores from the serious games and the severity of the disease using clinical assessment tools commonly used for FRDA. Results of patients were compared with a group a healthy subjects of similar age.ResultsVery highly significant differences were found for time required to perform the exercise (increase of 76%, t(68) = 7.22, P < 0.001) and for accuracy (decrease of 6%, t(68) = − 3.69, P < 0.001) between patients and healthy subjects. Concerning the patients significant correlations were found between age and time (R = 0.65, p = 0.015), accuracy (R = − 0.75, p = 0.004) and the total displacement of upper limbs. (R = 0.55, p = 0.031). Statistically significant correlations were found between the age of diagnosis and speed related parameters.ConclusionsThe results of this study indicate that SG reliably captures motor impairment of FRDA patients due to cerebellar and pyramidal involvement. Results also show that functional evaluation of FRDA patients can be performed during rehabilitation therapy embedded in games with the patient seated in a wheelchair.Trial registrationThe study was approved as a component of the EFACTS study (Clinicaltrials.gov identifier NCT02069509, registered May 2010) by the local institutional Ethics Committee (ref. P2010/132).
Highlights
Friedreich ataxia (FRDA) is a disease with neurological and systemic involvement
Clinical assessment tools commonly used for FRDA, such as the Scale for Assessment and Rating of Ataxia (SARA) [3], the Friedreich Ataxia Rating Scale (FARS) [4], and the International Cooperative Ataxia Rating Scale (ICARS) [5], become less effective in evaluating progression in patients with advanced FRDA, when they are in a wheelchair and when upper limb function is impaired
They were all enrolled in the European Friedreich Ataxia Consortium for Translational Studies (EFACTS, Clinicaltrials.gov identifier NCT02069509) natural history study, the first prospective pan-European FRDA registry designed to define clinical rating scales and quality of life measures, which can be used in clinical trials
Summary
Friedreich ataxia (FRDA) is a disease with neurological and systemic involvement. Clinical assessment tools commonly used for FRDA become less effective in evaluating decay in patients with advanced FRDA, when they are in a wheelchair. Clinical assessment tools commonly used for FRDA, such as the Scale for Assessment and Rating of Ataxia (SARA) [3], the Friedreich Ataxia Rating Scale (FARS) [4], and the International Cooperative Ataxia Rating Scale (ICARS) [5], become less effective in evaluating progression in patients with advanced FRDA, when they are in a wheelchair and when upper limb function is impaired This is partly due to a ceiling effect in these scales, in which gait and balance item have a major weight, and to the characteristics of the disease [6, 7]. The need for a more integrated tool to assess upper limb motor function in FRDA, in wheelchair-bound patients with advanced disease
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