The Use of New Mobile and Gaming Technologies for the Assessment and Rehabilitation of People with Ataxia: a Systematic Review and Meta-analysis.

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.

Predictive Validity of the Scale for the Assessment and Rating of Ataxia for Medium-Term Functional Status in Acute Ataxic Stroke.

OBJECTIVES: This study examines the prognostic validity of the Scale for the Assessment and Rating of Ataxia for patients with acute stroke. MATERIALS AND METHODS: We enrolled 120 patients with posterior circulation stroke having ischemic or hemorrhagic lesions with ataxia who had physical therapy. We recorded the clinical stroke features and obtained the scale for the assessment and rating of ataxia and National Institutes of Health Stroke Scale scores 7 days after admission and at discharge. Predictive factors for a 3-month modified Rankin Scale score of <3 were investigated. RESULTS: During hospitalization, the Scale for the Assessment and Rating of Ataxia score decreased from 7.5 (interquartile range, 4.5-12.5) to 4.0 (interquartile range, 1.5-8.0) points, whereas the National Institutes of Health Stroke Scale score changed from 1 (interquartile range, 0-3) to 1 (interquartile range, 0-2) point. A significant correlation between functional outcome and the Scale for the Assessment and Rating of Ataxia scores 7 days after onset was observed. The cutoff value for the assessment and rating of ataxia for predicting favorable outcome (modified Rankin scale, 0-2) at 3 months post-onset was 14 points (0-40) at 7 days after onset. CONCLUSIONS: The Scale for the Assessment and Rating of Ataxia scores showed good responsiveness to neurological changes in patients with acute ataxic stroke, could predict functional outcomes 3 months after onset on day 7, and could be a useful and reliable marker for patients with ataxic stroke.

Methotrexate-induced Acute Myelopathy in a Teenager With High-risk Acute Lymphoblastic Leukemia.

Acute lymphoblastic leukemia (ALL) is one of the most frequent malignancies in childhood whose long-term survival has increased up to 80% thanks to modern therapy enhancements. Nevertheless, methotrexate (MTX) remains a mainstay of ALL therapy, but also represents one of the major causes of neurotoxicity in patients with ALL. MTX-induced toxicity occurs in about 9% of patients treated for ALL. It usually affects deep white matter region leading to leukoencephalopathy, which has varying clinical manifestations ranging from acute neurologic disturbances to seizures or chronic permanent encephalopathy. Here we describe a 13-year-old girl affected with ALL who developed lower limbs hypesthesia and static ataxia due to transverse myelopathy after intrathec administration of MTX therapy. A high-dose corticotherapy combined to vitamin supplementation and rehabilitation was tested. Neurological evolution was characterized by slow and partial recovery.

Effect of object substitution, spontaneous compensation and repetitive training on reaching movements in a patient with optic ataxia.

We report the case of M.B. who demonstrated severe optic ataxia with the right hand following stroke in the left hemisphere. The clinical picture may shed light on both the pathological characteristics of reaching and grasping actions, and potential rehabilitation strategies for optic ataxia. First, M.B. demonstrated a dissociation between severely impaired reaching and relatively spared grasping and tool use skills and knowledge, which confirms that grasping may be more intermingled with non-motoric cognitive mechanisms than reaching. Besides, M.B.'s reaching performance was sensitive to movement repetition. We observed a substitution effect: Reaching time decreased if M.B. repeatedly reached toward the same object but increased when object identity changed. This may imply that not only object localization but also object identity, is integrated into movement programming in reach-to-grasp tasks. Second, studying M.B.'s spontaneous compensation strategies ascertained that the mere repetition of reaching movements had a positive effect, to the point M.B. almost recovered to normal level after an intensive one-day repetitive training session. This case study seems to provide one of the first examples of optic ataxia rehabilitation. Reaching skills can be trained by repetitive training even two years post-stroke and despite the presence of visuo-imitative apraxia.

Motor Function Improvement in Children with Ataxia Receiving Interval Rehabilitation, Including Vibration-Assisted Hometraining: A Retrospective Study.

BACKGROUND: Physiotherapy, including vibration-assisted therapy, has been proven to be effective for patients with ataxic cerebral palsy. Herewith, we studied the effect of a functional, goal-oriented interval rehabilitation program, including vibration-assisted home-training on the motor function of children with congenital ataxias. PATIENTS: 45 children (mean age 7.7 years, SD 4.70) with ataxia, having received a 6-month home-based side-alternating vibration-assisted therapy combined with intensive, goal-oriented, functional rehabilitation intervals, were included in the study, classified according to the progressive or non-progressive ataxia character. METHOD: Retrospective analysis of the prospectively collected data of the registry of the Cologne rehabilitation program "Auf die Beine". Motor abilities have been assessed prior to the intervention (M0), after 6 months of home-training (M6) as well as in a follow-up 6 months later (M12). We performed a gait analysis, a 1-minute walking test (1-MWT), and the Gross Motor Function Measure (GMFM-66). RESULTS: The GMFM-66 improvement (M6-M0 vs. M12-M6) was statistically significant with median improvement of 2.4 points (non-progressive) and 2.9 points (progressive) respectively, and clinically relevant. The 1-MWT improvement was statistically significant and clinically relevant for non-progressive ataxia. CONCLUSION: The intensive training, including vibration-assisted therapy significantly improved the motor function of children with ataxia. Six months later the skills were preserved in children with progressive ataxia and could be further developed in non-progressive forms.

Smart and Assistive Walker - ASBGo: Rehabilitation Robotics: A Smart-Walker to Assist Ataxic Patients.

Locomotion is an important human faculty that affects an individual's life, bringing not only physical and psychosocial implications but also heavy social-economic consequences. Thus, it becomes paramount to find means (augmentative/assistive devices) to empower the user's residual capacities and promote functional recovery.In this context, a smart walker (SW) is explored for further clinical evaluation of ataxic patients during walker-assisted and to serve as a functional compensation and assist-as-needed personalized/customized rehabilitation tool, autonomously adapting assistance to the users' needs, through innovative combination of real-time multimodal sensory information from SW built-in sensors. To meet the users' needs, its design was weighed, considering to whom it is intended.Thereby, this paper presents the system overview, focusing on design considerations, mechanical structure (frame and main components), electronic and mechatronic components, followed by its functionalities. Lastly, it presents results regarding the main functionalities, addressing clinical evidence.

Model for prompt and effective classification of motion recovery after stroke considering muscle strength and coordination factors.

BACKGROUND: Muscle synergies are now widely discussed as a method for evaluating the existence of redundant neural networks that can be activated to enhance stroke rehabilitation. However, this approach was initially conceived to study muscle coordination during learned motions in healthy individuals. After brain damage, there are several neural adaptations that contribute to the recovery of motor strength, with muscle coordination being one of them. In this study, a model is proposed that assesses motion based on surface electromyography (sEMG) according to two main factors closely related to the neural adaptations underlying motor recovery: (1) the correct coordination of the muscles involved in a particular motion and (2) the ability to tune the effective strength of each muscle through muscle fiber contractions. These two factors are hypothesized to be affected differently by brain damage. Therefore, their independent evaluation will play an important role in understanding the origin of stroke-related motor impairments. RESULTS: The model proposed was validated by analyzing sEMG data from 18 stroke patients with different paralysis levels and 30 healthy subjects. While the factors necessary to describe motion were stable across heathy subjects, there was an increasing disassociation for stroke patients with severe motor impairment. CONCLUSIONS: The clear dissociation between the coordination of muscles and the tuning of their strength demonstrates the importance of evaluating these factors in order to choose appropriate rehabilitation therapies. The model described in this research provides an efficient approach to promptly evaluate these factors through the use of two intuitive indexes.

The use of Goal Attainment Scaling (GAS) in the rehabilitation of ataxic patients.

The aim of this study was to formulate an exercise program according to GAS, an approach that reflects the patients' point of view and expectations and investigate the effects of this program in ataxia rehabilitation. This study was designed as an assessor-blinded, single-group trial, and 24 patients with cerebellar ataxia were included. Treatment goals, postural control, disease severity, and daily living activities were assessed pre- and post-treatment using the Goal Attainment Scaling (GAS), the Sensory Organization Test (SOT), the International Cooperative Ataxia Rating Scale (ICARS), and the Barthel Index (BI), respectively. An exercise program was applied by taking patients' individual expectations and treatment goals into account. The participants enrolled in the physiotherapy program for 8 weeks, 3 days a week for 1 hour per day. The mean age of the patients was 34.00 ± 9.15 years. While the GAS, kinetic functions ICARS subscores, ICARS total scores, SOT-composite, and BI scores improved significantly after treatment (p < 0.05), other ICARS subscores did not change after treatment (p > 0.05). The results showed that putting the patient at the center of the evaluation and treatment process while formulating a treatment plan had a positive effect on treatment outcome. If the functions that patients consider important are known and the treatment process concentrates on these functions, a patient's participation in his/her individual treatment is supported by increasing his/her motivation and contribution to more successful rehabilitation practices.

On the effect of walking surface stiffness on inter-limb coordination in human walking: toward bilaterally informed robotic gait rehabilitation.

BACKGROUND: Robotic devices have been utilized in gait rehabilitation but have only produced moderate results when compared to conventional physiotherapy. Because bipedal walking requires neural coupling and dynamic interactions between the legs, a fundamental understanding of the sensorimotor mechanisms of inter-leg coordination during walking, which are not well understood but are systematically explored in this study, is needed to inform robotic interventions in gait therapy. METHODS: In this study we investigate mechanisms of inter-leg coordination by utilizing novel sensory perturbations created by real-time control of floor stiffness on a split-belt treadmill. We systematically alter the unilateral magnitude of the walking surface stiffness and the timing of these perturbations within the stance phase of the gait cycle, along with the level of body-weight support, while recording the kinematic and muscular response of the uperturbed leg. This provides new insight into the role of walking surface stiffness in inter-leg coordination during human walking. Both paired and unpaired unadjusted t-tests at the 95 % confidence level are used in the approriate scernario to determine statistical significance of the results. RESULTS: We present results of increased hip, knee, and ankle flexion, as well as increased tibialis anterior and soleus activation, in the unperturbed leg of healthy subjects that is repeatable and scalable with walking surface stiffness. The observed response was not impacted by the level of body-weight support provided, which suggests that walking surface stiffness is a unique stimulus in gait. In addition, we show that the activation of the tibialis anterior and soleus muscles is altered by the timing of the perturbations within the gait cycle. CONCLUSIONS: This paper characterizes the contralateral leg's response to ipsilateral manipulations of the walking surface and establishes the importance of walking surface stiffness in inter-leg coordination during human walking.

Clinical usefulness and validity of robotic measures of reaching movement in hemiparetic stroke patients.

BACKGROUND: Various robotic technologies have been developed recently for objective and quantitative assessment of movement. Among them, robotic measures derived from a reaching task in the KINARM Exoskeleton device are characterized by their potential to reveal underlying motor control in reaching movements. The aim of this study was to examine the clinical usefulness and validity of these robot-derived measures in hemiparetic stroke patients. METHODS: Fifty-six participants with a hemiparetic arm due to chronic stroke were enrolled. The robotic assessment was performed using the Visually Guided Reaching (VGR) task in the KINARM Exoskeleton, which allows free arm movements in the horizontal plane. Twelve parameters were derived based on motor control theory. The following clinical assessments were also administered: the proximal upper limb section in the Fugl-Meyer Assessment (FMA-UE(A)), the proximal upper limb part in the Stroke Impairment Assessment Set (SIAS-KM), the Modified Ashworth Scale for the affected elbow flexor muscles (MAS elbow), and seven proximal upper limb tasks in the Wolf Motor Function Test (WMFT). To explore which robotic measures represent deficits of motor control in the affected arm, the VGR parameters in the paretic arm were compared with those in the non-paretic arm using the Wilcoxon signed rank test. Then, to explore which VGR parameters were related to overall motor control regardless of the paresis, correlations between the paretic and non-paretic arms were examined. Finally, to investigate the relationships between the robotic measures and the clinical scales, correlations between the VGR parameters and clinical scales were investigated. Spearman's rank correlation coefficients were used for all correlational analyses. RESULTS: Eleven VGR parameters on the paretic side were significantly different from those on the non-paretic side with large effect sizes (|effect size| = 0.76-0.87). Ten VGR parameters correlated significantly with FMA-UE(A) (|r| = 0.32-0.60). Eight VGR parameters also showed significant correlations with SIAS-KM (|r| = 0.42-0.49), MAS elbow (|r| = 0.44-0.48), and the Functional Ability Scale of the WMFT (|r| = 0.52-0.64). CONCLUSIONS: The robot-derived measures could successfully differentiate between the paretic arm and the non-paretic arm and were valid in comparison to the well-established clinical scales.

Balance exercise in patients with chronic sensory ataxic neuropathy: a pilot study.

Although exercise therapy is considered part of the treatment of neuropathic patients, and somatosensory input is essential for motor learning, performance and neural plasticity, rehabilitation of patients with sensory ataxia has received little attention so far. The aim of this prospective pilot study was to explore the short- and medium-term efficacy of a 3-week intensive balance and treadmill exercise program in chronic ataxic neuropathy patients; 20 consecutive patients with leg overall disability sum score (ODSS-leg) ≥2, absent/mild motor signs, clinical and therapeutic stability ≥4 months were enrolled. Evaluations were done at baseline, at the end of treatment and at 3- and 6-month follow-up. Outcome measurements included: ODSS-leg, Berg balance scale, 6-min walk distance, and the functional independence measure (FIM) scale. The short-form-36 health status scale (SF-36) was used to measure health-related quality of life (HRQoL). ODSS-leg improved significantly compared with baseline, 3 weeks, 3 months (primary outcome), and 6 months follow-up. A significant improvement in all functional secondary outcome measurements and in some SF-36 subscales was also observed. This pilot study suggests that balance exercise is safe and well tolerated and might be effective in ameliorating disability and HRQoL in patients with chronic peripheral sensory ataxia.

Effectiveness of hand splints in children with cerebral palsy: a systematic review with meta-analysis.

AIM: The aim of this review was to determine the effectiveness of hand splinting for improving hand function in children with cerebral palsy (CP) and brain injury. METHOD: A systematic review with meta-analyses was conducted. Only randomized and quasi-randomized controlled trials in which all participants were children aged 0 to 18 years with CP or brain injury and a hand splint (cast, brace, or orthosis) were included. RESULTS: Six studies met the inclusion criteria. No study included participants with a brain injury; therefore, the results relate only to CP. Five studies investigated 'non-functional hand splints' and one investigated a 'functional hand splint'. Moderate-quality evidence indicated a small benefit of non-functional hand splints plus therapy on upper limb skills over therapy alone (standard mean difference [SMD]=0.81, 95% confidence interval [CI]=0.03-1.58), although benefits were diminished 2 to 3 months after splint wearing stopped (SMD=0.35, CI -0.06 to 0.77). INTERPRETATION: In children with CP, hand splints may have a small benefit for upper limb skills. However, results are diminished after splint wearing stops. Given the costs - potential negative cosmesis and discomfort for the child - clinicians must consider whether hand splinting is clinically worthwhile. Further methodologically sound research regarding hand splinting combined with evidence-based therapy is needed to investigate whether the small clinical effect is meaningful.

Proprioception: where are we now? A commentary on clinical assessment, changes across the life course, functional implications and future interventions.

Proprioception, the sense of where one is in space, is essential for effective interaction with the environment. A lack of or reduction in proprioceptive acuity has been directly correlated with falls and with reduced functional independence in older people. Proprioceptive losses have also been shown to negatively correlate with functional recovery post stroke and play a significant role in other conditions such as Parkinson's disease. However, despite its central importance to many geriatric syndromes, the clinical assessment of proprioception has remained remarkably static. We look at approaches to the clinical assessment of proprioception, changes in proprioception across the life course, functional implications of proprioception in health and disease and the potential for targeted interventions in the future such as joint taping, and proprioception-specific rehabilitation and footwear.

[Results or pelvi-perineal rehabilitation in patients with pelvic floor dyssynergia]. / Resultados de la de rehabilitación pelviperineal en pacientes con disinergia del suelo pélvico.

INTRODUCTION: Pelvic floor dyssynergia (PPD) is a common cause of outlet obstruction constipation. Treatment for this condition is based on pelvi-perineal re-education (PPR). The aim of this study was to evaluate the results of PPR on patients with PPD METHODS: Patients with the diagnosis of PPD were included. The study was conducted between 2010 and 2011. PPR was performed by specialized kinesiologists. Prior and after treatment a constipation questionnaire was performed (KESS) (scale from 0 to 39 points, a higher score is associated with more symptom severity). KESS score before and after PPR were compared. Mann-Whitney-Wilcoxon rank sum test for paired samples was used for statistical analysis, p value <0,05 was considered as significant. RESULTS: Thirteen patients were included (11 women), mean age 44.3 years old (r: 18-76). Mean total KESS score prior and after PPR were 19.6 (SD: 5.8) and 12.6 (DS: 63), respectively (P=.002). Frequency of bowel movements, stool consistency, abdominal pain and abdominal bloating did not present statistically significant changes before and after treatment. Use of laxatives, enemas and/or digitations, as well as unsuccessful evacuation, feelings of incomplete evacuation improved significantly. Total evacuation time (before 1.53 vs after 1; P=.012) and difficult evacuation causing painful efforts (before 2.08 vs after 1.07; P=.001) also decreased significantly. CONCLUSION: PPR in patients with PPD, significantly improves the symptoms of obstructive constipation, mainly with respect to mechanical assistance and difficult evacuation.

Status epilepticus in a child secondary to ingestion of skin-lightening cream.

The popularity of the Internet and online media has led to the increased availability of prescription-strength, skin-lightening products contributing to a rise in their use among people with various skin pigment disorders. These products may contain a wide variety of active ingredients such as heavy metals, hydroquinone, and corticosteroids that can be highly toxic, especially after prolonged application. For decades, there have been case reports of both corticosteroid and heavy metal toxicity related to skin-lightening cream use. We report a case of a child who developed status epilepticus after ingesting a skin-lightening solution containing 2% hydroquinone. The toxicodynamics of hydroquinone and its effects on the central nervous system are discussed.

Discriminant validity of a new measure of self-efficacy for reaching movements after stroke-induced hemiparesis.

STUDY DESIGN: Case-control study. INTRODUCTION: After stroke, difficulties in motor control mediate spontaneous paretic arm use in real life. Along with the obvious motor impairments, self-efficacy has been shown to be an important modifier for physical function. However, a self-efficacy measure for arm reaching is lacking. The aim of this study was to develop such a measure and to examine its validity. METHODS: Fifteen participants with hemiparesis and ten control participants were recruited. A measure of reaching self-efficacy (RSE) was developed using a two-dimensional center-out target array. RESULTS: For the control group, RSE was significantly correlated with target distance (r = -0.657) and location (r = -0.545), respectively. Additionally, average RSE was lower for the non-dominant than the dominant hand (p < 0.001). More importantly, for the stroke group, RSE was significantly correlated with target distance (r = -0.603) and location (r = -0.378), respectively. Finally, average RSE was lower for the paretic than the non-paretic hand (p < 0.001). CONCLUSIONS: These results demonstrate robust discriminant validity for this new measure of reaching self-efficacy. LEVEL OF EVIDENCE: 3b.

A case of very rapid progressive ataxia in rehabilitation setting.

AIM: To describe the unusual course of a patient with cerebellar ataxia who underwent rehabilitation. METHOD: At admission, the patient underwent motor rehabilitation and assessment by Tinetti Assessment, Klockgether Score, National Institute of Health Stroke Scale, Trunk Control Test, Fugl-Meyer Scale, Barthel Index, and Mini Mental State Examination. RESULTS: The patient showed very rapid and severe decline of motor and functional tests during the first month of hospitalization and complete dependence in <2 months, despite rehabilitation. He died 10 months after onset, with a neuro-pathological diagnosis of sporadic Creutzfeldt-Jakob disease (CJD). CONCLUSION: Our case report suggests that sporadic CJD must be hypothesized when ataxia worsens rapidly and severely despite rehabilitation.

Effects of calisthenics and Pilates exercises on coordination and proprioception in adult women: a randomized controlled trial.

OBJECTIVE: To assess and compare the effects of 6 mo of Pilates and calisthenics on multijoint coordination and proprioception of the lower limbs at the 3rd and 6th mo of training. DESIGN: Randomized, controlled, assessor-blinded, repeated-measures. SETTING: University research laboratory. PARTICIPANTS AND INTERVENTION: Healthy, sedentary, female participants age 25-50 y were recruited and randomly divided into 3 groups: a calisthenic exercise group (n = 34, mean age ± SD 40 ± 8 y, body-mass index [BMI] 31.04 ± 4.83 kg/m2), a Pilates exercise group (n = 32, mean age ± SD 37 ± 8 y, BMI 31.04 ± 4.83 kg/m2), and a control group (n = 41, mean age ± SD 41 ± 7 y, BMI 27.09 ± 4.77 kg/m2). The calisthenics and Pilates groups underwent related training programs for 6 mo, while the controls had no specific training. MAIN OUTCOME MEASURES: Coordination and proprioception of the lower extremities with concentric and eccentric performances in the closed kinetic chain assessed with the monitored rehab functional squat system at baseline and at the 3rd and 6th mo of training. RESULTS: For the within-group comparison, coordinative concentric and eccentric deviation values were significantly decreased for both dominant and nondominant lower limbs at pretraining and at the 3rd and 6th mo posttraining in the calisthenics group (P < .05). In contrast, there was no improvement in the Pilates group throughout the training. However, for comparisons between groups, the baseline values of coordinative concentric and eccentric deviations were different in the calisthenics group than in Pilates and the controls (P < .05). There were no differences in the proprioception values of either visible or nonvisible movement in any group throughout the training (P > .05). CONCLUSIONS: It seems that calisthenic exercises are more likely to improve coordination of the lower extremity after 3 and 6 mo of training than Pilates exercises. Calisthenic exercises may be useful for individuals who require improved coordination.

Balint's syndrome and post-acute brain injury rehabilitation: a case report.

BACKGROUND: Balint's syndrome includes the clinical symptom triad of simultagnosia, ocular apraxia and optic ataxia. These symptoms, in combination, are rare and can be quite debilitating as they impact visuospatial skills, visual scanning and attentional mechanisms. CASE STUDY: The literature addressing rehabilitation of individuals with Balint's syndrome is sparse. The current case report describes the outcome of a 58-year old male who presented with Balint's syndrome secondary to severe traumatic brain injury and following completion of a comprehensive post-acute brain injury rehabilitation programme. The patient was 4-months post-injury onset upon admission and received 6 months of rehabilitation services as an inpatient. The patient's comprehensive rehabilitation programme involved a 3-pronged approach including the implementation of (a) compensatory strategies, (b) remediation exercises and (c) transfer of learned skills in multiple environments and situations with implementation of psychoeducation and psychotherapy. Comprehensive neuropsychological and occupational therapy evaluations were performed at admission and at discharge in order to monitor cognitive, affective, neurological and functional change over time. CONCLUSIONS: Neuropsychological test improvements were noted on tasks that assess visuospatial functioning, although most gains were noted for functional and physical abilities.