Neurorehabilitation therapy in spinocerebellar ataxia type 2: A 24‐week, rater‐blinded, randomized, controlled trial

Rodríguez-Díaz, Julio Cesar; Velázquez‐Pérez, Luis; Labrada, Roberto Rodríguez; Rodríguez, Raúl Aguilera; Pérez, Dalina Laffita; Ochoa, Nalia Canales; Montero, Jacqueline Medrano; Rodríguez, Annelié Estupiñán; Borjas, Marcos Osorio; Marrero, Mariela Góngora; Cejas, Lorenzo Reynaldo; Zaldívar, Yanetza González; Gotay, Dennis Almaguer

doi:10.1002/mds.27437

Cited by 43 publications

(40 citation statements)

References 41 publications

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“…At present, there is no disease-modifying therapy for SCAs, only the symptomatic treatment and the methods of a palliative care are used to support SCA patients [120]. A recent 24-week, rater-blinded, randomized, controlled trial on SCA2 patients revealed that the tested rehabilitation program significantly improved the SARA scores of SCA2 patients likely as a result of the partial preservation of the motor learning and neural plasticity mechanisms suggesting that palliative treatment does have an actual effect in the treatment of SCA2 patients [121]. Current clinical studies show that some ataxia signs can be partly improved pharmacologically by treatment with 4-aminopyridine, riluzole, valproic acid, and thyrotropinreleasing hormone, whereas lithium and deferiprone have a negative effect or its absence [122].…”

Section: Ataxiamentioning

confidence: 99%

Molecular Mechanisms and Therapeutics for Spinocerebellar Ataxia Type 2

Egorova

Bezprozvanny

2019

Neurotherapeutics

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The effective therapeutic treatment and the disease-modifying therapy for spinocerebellar ataxia type 2 (SCA2) (a progressive hereditary disease caused by an expansion of polyglutamine in the ataxin-2 protein) is not available yet. At present, only symptomatic treatment and methods of palliative care are prescribed to the patients. Many attempts were made to study the physiological, molecular, and biochemical changes in SCA2 patients and in a variety of the model systems to find new therapeutic targets for SCA2 treatment. A better understanding of the uncovered molecular mechanisms of the disease allowed the scientific community to develop strategies of potential therapy and helped to create some promising therapeutic approaches for SCA2 treatment. Recent progress in this field will be discussed in this review article.

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Section: Ataxiamentioning

confidence: 99%

Molecular Mechanisms and Therapeutics for Spinocerebellar Ataxia Type 2

Egorova

Bezprozvanny

2019

Neurotherapeutics

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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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“…Cuba is the country with the world's highest prevalence of SCA2 (28 mutation carriers per 10 inhabitants), particularly in the province of Holguín, where it reaches around 182 mutation carriers per 10 inhabitants as a result of a founder effect …”

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confidence: 99%

“…To date, therapies for SCA2 are largely symptomatic, controlling symptoms rather than mitigating disease progression . However, neurorehabilitation approaches have shown significant benefits for motor and nonmotor features of the disease …”

mentioning

confidence: 99%

“…Taking into account that neurorehabilitation is a safe and effective therapeutic option for SCA2 patients, together with the known cerebellar involvement in prodromal stage, we tested the feasibility of a neurorehabilitation program in the SCA2 prodromal disease stage by means of a 12‐week, rater‐blinded, randomized, controlled clinical trial in 30 nonataxic SCA2 mutation carriers. This is the first preclinical intervention in a homogeneous population of SCAs performed worldwide.…”

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confidence: 99%

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

et al. 2019

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Background The search for early interventions is a novel approach in spinocerebellar ataxias, but there are few studies supporting this notion. This article aimed to assess the efficacy of neurorehabilitation treatment in prodromal spinocerebellar ataxia type 2. Methods Thirty spinocerebellar ataxia type 2 preclinical carriers were enrolled in a randomized, controlled trial using neurorehabilitation. The intervention in the treated group was 4 hours per day, 5 days per week for 12 weeks, emphasizing static balance, gait, and limb coordination. The control group did not receive rehabilitation. The primary outcome measure was the time for 5‐m tandem gait over the floor. Secondary outcomes included other timed tests with increased motor complexity, as well as the scores of the SARA and the Inventory of Non‐ataxia Symptoms. Results The times for 5‐m tandem gait over the floor and the mattress were significantly reduced only in the rehabilitated group. Moreover, the times upholding the tandem stance over a mattress and the seesaw were notably increased only in this group. Likewise, the finger‐nose and the heel‐shin tests were improved in the rehabilitated group alone. The SARA score and the count of nonataxia symptoms were unchanged. Conclusions This rehabilitation program improves the subtle gait, postural and coordinative deficits in prodromal spinocerebellar ataxia type 2, which provided novel hints about the preservation of motor learning and neural plasticity mechanisms in early disease stages, leading chances for other interventional approaches in this and other spinocerebellar ataxias. © 2019 International Parkinson and Movement Disorder Society

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Neurorehabilitation therapy in spinocerebellar ataxia type 2: A 24‐week, rater‐blinded, randomized, controlled trial

Cited by 43 publications

References 41 publications

Molecular Mechanisms and Therapeutics for Spinocerebellar Ataxia Type 2

Molecular Mechanisms and Therapeutics for Spinocerebellar Ataxia Type 2

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

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

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