***TASK*** the task is to summarize an input biomedical literature in six sentences 
    ***INPUT*** the input is a biomedical literature 
    ***OUTPUT*** the output is the summary of an input biomedical literature in six sentences 
    ***DOCUMENTATION*** 
    ***EXAMPLES*** 
    Input: BACKGROUND AND PURPOSE A pilot study of high dose coenzyme Q(10 ) ( CoQ(10))/vitamin E therapy in Friedreich 's ataxia ( FRDA ) patients result ed in significant clinical improvements in most patients . This study investigated the potential for this treatment to modify clinical progression in FRDA in a r and omized double blind trial . METHODS Fifty FRDA patients were r and omly divided into high or low dose CoQ(10)/ vitamin E groups . The change in International Co-operative Ataxia Ratings Scale ( ICARS ) was assessed over 2 years as the primary end-point . A post hoc analysis was made using cross-sectional data . RESULTS At baseline serum CoQ(10 ) and vitamin E levels were significantly decreased in the FRDA patients ( P < 0.001 ) . During the trial CoQ(10 ) and vitamin E levels significantly increased in both groups ( P < 0.01 ) . The primary and secondary end-points were not significantly different between the therapy groups . When compared to cross-sectional data 49 % of all patients demonstrated improved ICARS scores . This responder group had significantly lower baseline serum CoQ(10 ) levels . CONCLUSIONS A high proportion of FRDA patients have a decreased serum CoQ(10 ) level which was the best predictor of a positive clinical response to CoQ(10)/vitamin E therapy . Low and high dose CoQ(10)/vitamin E therapies were equally effective in improving ICARS scores Background : Friedreich ’s ataxia ( FRDA ) , the most common genetic cause of ataxia , is characterised by progressive neurodegeneration and cardiomyopathy . Initial treatments are likely to slow progression rather than reverse morbidity . An appropriate and sensitive scale to measure disease progress is critical to detect the benefit of treatments . Objective : To compare the  Friedreich Ataxia Rating Scale ( FARS )  with other scales proposed as outcome measures for FRDA . Methods : 76 participants were assessed with the  FARS  and the  International Cooperative Ataxia Rating Scale ( ICARS )  and 72 of these participants were also assessed with the  Functional Independence Measure  and the  Modified Barthel Index .  43 participants had repeat measures at an interval of 12 months . Sensitivity and responsiveness were assessed using the effect size for each measure and the sample size required for a placebo-controlled clinical trial . Results : The FARS showed a high correlation with the other three measures . A significant change in the score over 12 months was detected by the FARS , the International Cooperative Ataxia Rating Scale and the Functional Independence Measure . The FARS had the greatest effect size and requires fewer patients for an equivalently powered study . Conclusions : Of the scales assessed , the FARS is the best to use in clinical trials of FRDA . This is based on effect size , and power calculations that show that fewer participants are required to demonstrate the same effect of an intervention . Further work is required to develop more sensitive and responsive instruments Objective : To examine the potential validity of performance measures and examination-based scales in Friedreich ataxia ( FA ) by examining their correlation with disease characteristics . Methods : The authors assessed the properties of a c and i date clinical outcome measure , the  Friedreich Ataxia Rating Scale ( FARS )  , and simple performance measures ( 9-hole peg test , the timed 25-foot walk , PATA test , and low-contrast letter acuity )  in 155 patients with FA from six institutions , and correlated the scores with disease duration , functional disability , activity of daily living scores , age , and shorter GAA repeat length to assess whether these measures capture the severity of neurologic dysfunction in FA . Results : Scores for the FARS and performance measures correlated significantly with functional disability , activities of daily living scores , and disease duration , showing that these measures meet essential criteria for construct validity for measuring the progressive nature of FA . In addition , the FARS and transformed performance measures scores were predicted by age and shorter GAA repeat length in linear regression models accounting for sex and testing site . Correlations between performance measures were moderate in magnitude , suggesting that each test captures separate yet related dimensions of neurologic function in FA and that a composite measure might better predict disease status . Composite measures created using cohort means and st and ard deviations predicted disease status better than or equal to single performance measures or examination-based measures . Conclusions : The Friedreich Ataxia Rating Scale , performance measures , and performance measure composites provide valid assessment s of disease progression in Friedreich ataxia Background : The pleiotropic effects of riluzole may antagonize common mechanisms underlying chronic cerebellar ataxia , a debilitating and untreatable consequence of various diseases . Methods : In a r and omized , double-blind , placebo-controlled pilot trial , 40 patients presenting with cerebellar ataxias of different etiologies were r and omly assigned to riluzole ( 100 mg/day ) or placebo for 8 weeks . The following outcome measures were compared : proportion of patients with a decrease of at least 5 points in the International Cooperative Ataxia Rating Scale ( ICARS ) total score after 4 and 8 weeks compared with the baseline score ; mean changes from the baseline to posttreatment ICARS ( total score and subscores at 8 weeks ) ; and occurrence of adverse events . Results : Riluzole and placebo groups did not differ in baseline characteristics . The number of patients with a 5-point ICARS drop was significantly higher in the riluzole group than in the placebo group after 4 weeks ( 9/19 vs 1/19 ; odds ratio [ OR ] = 16.2 ; 95 % confidence interval [ CI ] 1.8–147.1 ) and 8 weeks ( 13/19 vs 1/19 ; OR = 39.0 ; 95 % CI 4.2–364.2 ) . The mean change in the riluzole group ICARS after treatment revealed a decrease ( p < 0.001 ) in the total score ( −7.05 [ 4.96 ] vs 0.16 [ 2.65 ] ) and major subscores ( −2.11 [ 2.75 ] vs 0.68 [ 1.94 ] for static function , −4.11 [ 2.96 ] vs 0.37 [ 2.0 ] for kinetic function , and −0.74 [ 0.81 ] vs 0.05 [ 0.40 ] for dysarthria ) . Sporadic , mild adverse events occurred . Conclusions : These findings indicate the potential effectiveness of riluzole as symptomatic therapy in diverse forms of cerebellar ataxia . Classification of evidence : This study provides Class I evidence that riluzole reduces , by at least 5 points , the ICARS score in patients with a wide range of disorders that cause cerebellar ataxia ( risk difference 63.2 % , 95 % CI 33.5%–79.9 % ) OBJECTIVE To assess the efficacy of idebenone on neurological function in patients with Friedreich ataxia . DESIGN R and omized , double-blind , placebo-controlled intervention trial . SETTING Children 's Hospital of Philadelphia and the University of California at Los Angeles . PARTICIPANTS Seventy ambulatory pediatric patients ( age , 8 - 18 years ) with a baseline International Cooperative Ataxia Rating Scale ( ICARS ) score of 10 to 54 . INTERVENTIONS Participants were r and omized into 1 of 3 treatment arms : 450 or 900 mg of idebenone per day ( in those with a body weight < or = or > 45 kg , respectively ; n = 22 ) ; 1350 or 2250 mg of idebenone per day ( n = 24 ) ; or placebo ( n = 24 ) . MAIN OUTCOME MEASURES Mean change from baseline to week 24 in ICARS score was the primary efficacy variable . Mean change in Friedreich Ataxia Rating Scale ( FARS ) score , performance measures , and activities of daily living were the secondary efficacy variables . RESULTS Patients who received idebenone improved by 2.5 points on mean ICARS score compared with baseline , while patients in the placebo group improved by 1.3 points . Patients who took idebenone also improved by 1.6 points on the FARS , while patients taking placebo declined by 0.6 points . For both end points , the difference between the idebenone and placebo groups was not statistically different . CONCLUSIONS Idebenone did not significantly alter neurological function in Friedreich ataxia during the 6-month study . Larger studies of longer duration may be needed to assess the therapeutic potential of drug c and i date s on neurological function in Friedreich ataxia . Trial Registration clinical trials.gov Identifier : NCT00537680 Objective – To preliminarily compare the efficacy of pregabalin with that of placebo on the cerebellar signs caused by cortical cerebellar atrophy ( CCA ) . A deficiency of gamma‐aminobutyric acid ( GABA ) has been described in the cerebellum in CCA , and pregabalin has been shown to enhance GABA release in rat hippocampus BACKGROUND Olivopontocerebellar atrophy ( OPCA ) is a chronic neurodegenerative disease with symptoms of cerebellar ataxia , parkinsonism , autonomic disturbances and ophthalmoplegia . Buspirone , a 5-HT1(A ) agonist could constitute a symptomatic improvement in cerebellar dysfunction whereas estrogen has been investigated for neuroprotection . We conducted an open-labeled pilot trial to assess the efficacy of estrogen with buspirone treatment . PATIENTS AND METHODS Eighteen patients ( 7 male and 11 female ) with OPCA were r and omized into the buspirone ( 15 mg/day , n=9 ) , or the combined treatment group ( estrogen , 0.625 mg/d plus buspirone , n=9 ) . For the clinical rating , International Cooperative Ataxia Rating Scale ( ICARS ) was used and dysarthria , gaze evoked nystagmus , finger to nose , pronation-supination alternating movement , knee-tibia test , and gait speed were evaluated for 12 months . RESULTS Buspirone-treated group showed improvements in finger to nose and pronation-supination alternating movement test ( p=0.046 and p=0.025 , respectively ) . The combination group ( Estrogen+buspirone ) , however , showed no improvement in cerebellar sub-scales compared to the baseline . CONCLUSIONS Buspirone treatment showed feasible efficacies for OPCA , while the combined treatment of estrogen and buspirone failed to improve , suggesting estrogen may not have further benefit in cerebellar dysfunction Abstract . The aim of this study was to assess the efficacy and the safety of ondansetron administered orally in patients with a cerebellar disorder . The study was a r and omised , multi-center , double-blind trial . The patients were r and omised either to oral ondansetron 8 mg or to placebo twice daily for seven days . Cerebellar dysfunction was quantified before and after treatment using the International Cooperative Ataxia Rating Scale ( ICARS ) . We performed a global analysis ( total scores ) , we analysed by subscores ( 4 subscores : oculomotor , speech , kinetic , postural ) and subgroups ( 4 subgroups : Cerebellar Cortical Atrophy ( CCA ) , Multiple Systemic Atrophy ( MSA ) , Familial Cerebellar Degeneration ( FCD ) and miscellaneous cerebellar disorders ) , and we also performed an analysis by individual test items . We investigated whether ondansetron and placebo had different effects upon ICARS total scores and subscores in the 4 subgroups considered together or separately . For p values < 0.05 , we subsequently applied the Mann-Whitney test to compare ondansetron and placebo effect for each individual item . We evaluated 45 of the 46 patients included . No effect was found in global analysis . We found no difference in the analysis of the ICARS subscores . Concerning the individual test items , there was a significant difference between the placebo and ondansetron for the finger-to-nose test ( p = 0.049 ) , the Heel-to-Knee test ( HK ) ; ( p = 0.03 ) , the Body Sway Eyes Closed ( p = 0.017 ) and the Body Sway Eyes Open ( BSEO ) ; ( p = 0.014 ) . There was no significant difference for tremor in upper limbs ( p = 0.32 ) or for gait ( p = 0.49 ) . The Mann-Whitney test showed a greater effect of ondansetron than placebo for BSEO in miscellaneous disorders ( p = 0.013 ) and for HK in FCD ( p = 0.036 ) , but ondansetron was deleterious for HK in CCA ( p = 0.019 ) . Our study showed no effect of oral ondansetron on global cerebellar dysfunction . The analysis by subgroups showed that the oral form of ondansetron ( a ) is deleterious for coordination in patients with CCA , ( b ) has no effect upon tremor in upper limbs , and ( c ) has a mild effect upon posture and coordination in lower limbs in some subgroups of ataxic diseases BACKGROUND Friedreich 's ataxia ( FA ) is a progressive , multisystem , degenerative disorder caused by a reduction in frataxin . Loss of frataxin results in mitochondrial dysfunction and oxidative damage in patients and model systems . Previous studies have indicated that the antioxidant idebenone ( 5 mg/kg daily ) reduces cardiac hypertro 
    Output:
    
SARA and ICARS were the best studied and vali date d so far , and their reliability sustain their use .
Ataxia and non-ataxia scores will probably provide a better view of the overall disability in long-term trials and studies of natural history .