Neurorehabilitation Improves the Motor Features in Prodromal SCA2: A Randomized, Controlled Trial

Velázquez‐Pérez, Luis; Rodríguez-Díaz, Julio Cesar; Rodríguez‐Labrada, Roberto; Medrano‐Montero, Jacqueline; Cruz, Annety B. Aguilera; Reynaldo-Cejas, Lorenzo Enrique; Góngora‐Marrero, Mariela; Estupiñán‐Rodríguez, Annelié; Vázquez‐Mojena, Yaimeé; Torres‐Vega, Reidenis

doi:10.1002/mds.27676

Cited by 14 publications

(16 citation statements)

References 41 publications

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“…Overall, the identification of subtle quantitative deficits in gait and postural sway can impact positively in the clinical contexts because it guides the clinical decision‐making for the inclusion of pre‐SCA2 individuals in available early interventional programs, such as physical therapy 40 and further clinical trials of neuroprotective drugs. In fact, the identification of several quantitative variables with large and very large effect sizes suggests that their use as outcome measures could reduce the necessary sample size for future clinical trials.…”

Section: Discussionmentioning

confidence: 99%

Prodromal Spinocerebellar Ataxia Type 2 Subjects Have Quantifiable Gait and Postural Sway Deficits

et al. 2020

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Background The search for valid preclinical biomarkers of cerebellar dysfunction is a key research goal for the upcoming era of early interventional approaches in spinocerebellar ataxias. This study aims to describe novel preclinical biomarkers of subtle gait and postural sway abnormalities in prodromal spinocerebellar ataxia type 2 (pre‐SCA2). Methods Thirty pre‐SCA2 patients and their matched healthy controls underwent quantitative assessments of gait and postural sway using a wearable sensor‐based system and semiquantitative evaluation of cerebellar features by SARA (Scale for the Assessment and Rating of Ataxia) score. Results Quantitative analysis of natural gait showed a significantly larger variability of the swing period, toe‐off angle and toe‐out angle in pre‐SCA2, and larger mean coronal and transverse ranges of motion of the trunk at the lumbar location and of the sagittal range of motion of the trunk at the sternum location compared to controls. During tandem gait, pre‐SCA2 subjects showed larger lumbar, trunk, and arm ranges of motion than controls. Postural sway analysis showed excessive body oscillation that was increased in tandem stance. Overall, these abnormalities were detected in pre‐SCA2 patients without clinical evidence of abnormalities in SARA. The toe‐off angle and swing time variability were significantly correlated with the time to ataxia onset, whereas the toe‐off angle and transverse range of motion at trunk position during tandem gait were significantly associated with the SARA score. Conclusions This study demonstrates early alteration of gait and postural sway control in prodromal SCA2 using a wearable sensor‐based system. This offers new pathophysiological hints into this early disease stage and provides novel potential biomarkers for future clinical trials. © 2020 International Parkinson and Movement Disorder Society

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Section: Discussionmentioning

confidence: 99%

Prodromal Spinocerebellar Ataxia Type 2 Subjects Have Quantifiable Gait and Postural Sway Deficits

et al. 2020

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“…One of the main body function disorders in SCA that should be addressed is balance and postural control [9,17]. Consistently studies had shown that balance is disturbed both during static and dynamic, hence implies difficulty in performing effective gait, and maintaining standing position [9,18,19]. Since cerebellum also becomes a relay center for agonist and antagonistic complimentary contractions, it is natural to see that spinocerebellum lesion would affect effective voluntary muscle contractions, which could also present as central hypotonia [4,6].…”

Section: Body Structure and Body Functionmentioning

confidence: 99%

“…Majority of the published studies have mentioned how physiotherapy plays a big role in mobility interventions of SCA patients, be it through conventional therapy or through exergames and virtual reality [9]. Since physiotherapy intervention is primarily focused on achieving better gait control, it would naturally revolve on improving balance, strength, endurance, and posture simultaneously [18,27]. This finding has resulted into a more focused exercise sessions in the recent studies, aiming mainly on trunk balance, as these would be positively reflected in significant improvement of Scale for the Assessment and Rating of Ataxia (SARA) score [19,38].…”

Section: Physiotherapy Interventionsmentioning

confidence: 99%

“…Although there are no published guidelines on SCA mobility exercise, several published studies from Cuban Centre for the Research and Rehabilitation of Hereditary Ataxia (CIRAH) had effectively shown the benefits of intensive neurorehabilitation as they have conducted [18,27]. The whole therapy lasted for four hours per day, five days per week, lasting for 12 weeks in total, hence to the authors' knowledge, this is the longest exercise duration seen per day.…”

Section: Physiotherapy Interventionsmentioning

confidence: 99%

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International Classification of Functioning, Health and Disability (ICF) Conceptual Approach towards Spinocerebellar Ataxia

Triangto¹,

Setiono²,

Purba³

2022

Spinocerebellar Ataxia - Concepts, Particularities and Generalities

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Spinocerebellar Ataxia (SCA) is an autosomal dominant disease with progressive decline towards functional capacity. Although studies had shown that there are various SCA types, physical medicine and rehabilitation approach would focus mostly on functional aspects in each individuals. Analysis through International Classification of Functioning, Disability, and Health would assist clinicians to identify activity and participation aspects of SCA, mostly revolves around mobility function. Good correlation of mobility with quality of life was also reported, and thus it is only natural that this becomes the main focus of rehabilitative intervention. Approximately one hour physical exercise session focusing on postural control and balance was proven to be effective in improving disease related measurement tool, functional capacity, and quality of life. These benefits could be improved through newer therapies such as exercise games and virtual reality, virtually creates a rapidly changing environment, thus providing training through anticipatory actions. It is speculated that neuroplasticity through self-recognition of errors are the main physiology of recovery in SCA. Finally, it could be seen that rehabilitation intervention remains to be a cornerstone in current ataxia therapy, with goals of achieving exercise gains while alleviating the natural functional decline of the disease.

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“…Studies have shown that rehabilitation training may improve the balance function of SCA patients [14][15][16] , but there is no consistent recommendation for rehabilitation training. It is therefore necessary to evaluate postural stability to optimise rehabilitation programmes for SCA3 patients to improve their function, reduce the risk of falling and improve their quality of life.…”

mentioning

confidence: 99%

An observational study of balance and proprioception function in patients with spinocerebellar ataxias type 3

Liu¹,

Liu²,

Xu³

et al. 2020

Preprint

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BackgroundPostural instability is one of the most disabling features of spinocerebellar ataxias type 3 (SCA3) and often leads to falls that reduce mobility and functional capacity. This study aimed to quantitatively analyse static and dynamic balance and proprioception function on postural control in patients with SCA3 using the Pro-kin system and optimise rehabilitation programmes for them.MethodsEight-one clinically diagnosed SCA3 patients (38 women, 43 men; aged 39.00 ± 9.66) and 62 healthy controls were studied and evaluated using the Pro-kin system (PK254P, Tecnobody S.r.l, Dalmine, Italy). The measurements included (1) a static balance test in two visual feedback conditions: eyes open (EO) and eyes closed (EC); (2) a dynamic balance test measuring limits of stability (LOS); and (3) a proprioception function test to obtain proprioceptive measurements on a multiaxial balance evaluator for both right and left lower limbs.ResultsCompared to controls, SCA3 patients showed significantly higher values of all static balance outcome variables with eyes open and eyes closed, implying postural instability. SCA3 patients showed significantly higher values in the standard deviation of body sway along the medio-lateral (ML) axis and in the velocity of body sway along the anterior-posterior (AP) axis. The overall scores and the scores for all eight LOS components were significantly lower in the SCA3 patients than in the controls. The mean values of AP index (API), ML index (MLI), Stability index (SI) and average trace error (ATE) were significantly greater in SCA3 patients compared to HC subjects, while API showed a trend toward higher values.ConclusionsSCA3 patients have a significant postural control disorder, and are likely to fall on the AP plane and prefer performing postural adjustments in the ML direction; a decreased proprioception function in the knee and ankle is also evident. Visual cues and proprioception should be emphasized in balance rehabilitation training. Attention should also be paid to improve muscle strength and range of motion.Trial registrationThe Chinese clinical test registration center. ChiCTR1800020133. Registered 15 december 2018 - Retrospectively registered, http://www.chictr.org.cn/showprojen.aspx?proj=33950

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Neurorehabilitation Improves the Motor Features in Prodromal SCA2: A Randomized, Controlled Trial

Cited by 14 publications

References 41 publications

Prodromal Spinocerebellar Ataxia Type 2 Subjects Have Quantifiable Gait and Postural Sway Deficits

Prodromal Spinocerebellar Ataxia Type 2 Subjects Have Quantifiable Gait and Postural Sway Deficits

International Classification of Functioning, Health and Disability (ICF) Conceptual Approach towards Spinocerebellar Ataxia

An observational study of balance and proprioception function in patients with spinocerebellar ataxias type 3

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