Active clinical trials for "Spinocerebellar Degenerations"

In individuals with spino-cerebellar atrophy (SCA), the delayed onset of antagonist muscle firing has been reported to be the cause of hypermetria. Hypermetria is a common deficit in individuals with spino-cerebellar atrophy SCA when they perform ballistic goal-directed movement. Based on the previous studies, ballistic goal-directed movements are controlled by a triphasic pattern of agonistic and antagonistic muscle activation. The origin of the EMG pattern is a central program, whereas the delayed onset of antagonistic muscle firing has been reported to be the cause of hypermetria. To develop a therapy method, the difference in temporal pattern and intensity of supraspinal excitability of agonist and antagonist bursts between healthy adults and individuals with SCA when performing rapid and slow goal-directed movements should be further investigated. Traditional rehabilitations of individuals with cerebellum lesion were limited to improve the functional performance of movement. Since the deficits of the goal-directed movement are at pre-movement programming, only feedforward training will be possible to re-establish an appropriate program. Previous showed that peripheral stimulation resulted in a facilitation of motor cortex. Our group also found that this facilitation in individuals with SCA was similar to the ones without SCA. Therefore, it is possible to adjust the control pattern of supraspinal excitability of agonist and antagonist busts of SCA patient with passively providing electrical stimulation contains normal control pattern of healthy human. The present study sought to investigate the difference in temporal pattern and intensity of supraspinal excitability of agonist and antagonist bursts between healthy adults and individuals with SCA when performing rapid and slow goal-directed movements.

This research study is testing body-worn sensors to measure movement during simple tests of coordination, in order to evaluate the progression and severity of ataxia.

The purpose of this study is to gather information on the possible symptoms that patients with atypical neuronal ceroid lipofuscinosis type 2 (also known as aTPP1 or atypical tripeptidyl peptidase deficiency) have and how they change over time.

Investigators expect there will be improvement in walking speed and steadiness after taking Dalfampridine, thereby improving activities of daily living and enhancing social and occupational functions for patients with spinocerebellar ataxia.

This is a randomized, double-blind, placebo-controlled Phase 2 study evaluating oral administration of CAD-1883 in the treatment of adults with a genotypic diagnosis of Spinocerebellar Ataxia (SCA). This study offers the opportunity to understand the safety, tolerability, and efficacy of CAD-1883 in the SCA patient population.

DESIGN: Pilot, Phase II, double-blind, placebo-controlled study JUSTIFICATION: In the literature one does not find a pharmacological treatment that changes the natural history of Spinocerebellar ataxtia type 3 (SCA3). Patients with this disease invariably become dependent. OBJECTIVES I. To determine safety and tolerability of phenylbutyrate in patients with SCA3. II. To provide early subsidies on the efficacy of phenylbutyrate in SCA3. DURATION: 12 months of a double-blind study. PLACE OF REALIZATION: Hospital de Clínicas de Porto Alegre, Brazil. NUMBER OF PATIENTS: 20 patients. CONCOMITANT MEDICATIONS: There are no concomitant medications that are prohibited unless they affect safety parameters of this study (hemogram and platelets; fasting serum glucose, AST, ALT, Gamma-GT, Bilirubins, Prothrombin time, Creatinine, Urea, Na, K, chlorides and arterial gasometry; electrocardiogram and echocardiogram). MEDICATIONS UNDER INVESTIGATION: Powdered sodium phenylbutyrate in sachets containing each 3g. At the start of the study, the dose will be 15g/day (five sachets) and may be reduced in case of mild adverse events. OUTCOMES Primary safety outcome: The number of adverse events, interruptions and dose reductions in the two groups (cases and controls). Efficacy outcomes: Efficacy outcomes are the following scores in both groups: NESSCA, SARA, Barthel, BDI, and WHOQol.

This study will test laryngeal adaptation in speech and swallowing function in healthy adults, in patients with cerebral stroke, and in patients with spinocerebellar ataxia type 6. The findings from this proposal will be the first step toward implementing rehabilitation techniques that help patients to prevent speech and swallowing errors before they occur.

The purpose of this study is to evaluate the safety, efficacy, and pharmacokinetics of KPS-0373 in SCD patients (Experience of clinical trials of KPS-0373)

The purpose of this study is to evaluate the long-term safety, efficacy, and pharmacokinetics of KPS-0373 in SCD patients

Spinocerebellar atrophy is the most common autosomal dominant inherited ataxia. There are over thirty subtypes, which characterize neurologic features differently. They all have obvious substantial cerebellar atrophies in image, and unstable gait、ataxia. In general a prevalence of about three cases per 100 000 people is assumed, but this may be an underestimate. Progressive neurologic degeneration, in about 10-20 years, will leads to disability or wheelchair-dependent. Accompanying with fatigue, downhill course of the disease often made patients depressive and hopeless. The recent review of researches concludes no effective therapy for the disease. The purpose of the investigator's study is to explore the Tai-chi exercise effect for spinocerebellar ataxia.