***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: Parkinson plus diseases , comprising mainly progressive supranuclear palsy ( PSP ) and multiple system atrophy ( MSA ) are rare neurodegenerative conditions . We design ed a double-blind r and omized placebo-controlled trial of riluzole as a potential disease-modifying agent in Parkinson plus disorders ( NNIPPS : Neuroprotection and Natural History in Parkinson Plus Syndromes ) . We analysed the accuracy of our clinical diagnostic criteria , and studied prognostic factors for survival .  Patients with an akinetic-rigid syndrome diagnosed as having PSP or MSA according to modified consensus diagnostic criteria were considered for inclusion . The psychometric validity ( convergent and predictive ) of the NNIPPS diagnostic criteria were tested prospect ively by clinical and pathological assessment s. The study was powered to detect a 40 % decrease in relative risk of death within PSP or MSA strata . Patients were r and omized to riluzole or matched placebo daily and followed up to 36 months . The primary endpoint was survival . Secondary efficacy outcomes were rates of disease progression assessed by functional measures .  A total of 767 patients were r and omized and 760 qualified for the Intent to Treat ( ITT ) analysis , stratified at entry as PSP ( 362 patients ) or MSA ( 398 patients ) . Median follow-up was 1095 days ( range 249–1095 ) . During the study , 342 patients died and 112 brains were examined for pathology . NNIPPS diagnostic criteria showed for both PSP and MSA excellent convergent validity with the investigators ’ assessment of diagnostic probability ( point-biserial correlation : MSA rpb = 0.93 , P < 0.0001 ; PSP , rpb = 0.95 , P < 0.0001 ) , and excellent predictive validity against histopathology [ sensitivity and specificity ( 95 % CI ) for PSP 0.95 ( 0.88–0.98 ) and 0.84 ( 0.77–0.87 ) ; and for MSA 0.96 ( 0.88–0.99 ) and 0.91 ( 0.86–0.93 ) ] . There was no evidence of a drug effect on survival in the PSP or MSA strata ( 3 year Kaplan – Meier estimates PSP-riluzole : 0.51 , PSP-placebo : 0.50 ; MSA-riluzole : 0.53 , MSA-placebo : 0.58 ; P = 0.66 and P = 0.48 by the log-rank test , respectively ) , or in the population as a whole ( P = 0.42 , by the stratified-log-rank test ) . Likewise , rate of progression was similar in both treatment groups . There were no unexpected adverse effects of riluzole , and no significant safety concerns . Riluzole did not have a significant effect on survival or rate of functional deterioration in PSP or MSA , although the study reached over 80 % power to detect the hypothesized drug effect within strata . The NNIPPS diagnostic criteria were consistent and valid . They can be used to distinguish between PSP and MSA with high accuracy , and should facilitate research into these conditions relatively early in their evolution 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 % ) We conducted a r and omized double‐blind trial of  riluzole in Huntington 's disease to investigate the efficacy of this antiexcitotoxic drug in slowing disease progression Background : Riluzole retards striatal glutamate release and pathologic consequences in neurotoxic animal models of Huntington ’s disease ( HD ) . Objective : To determine the dosage-related impact of riluzole on chorea in HD . Methods : An 8-week double-blind dose-ranging multicenter study of riluzole was conducted in 63 subjects ( 32 women , 31 men ) with HD who were r and omized to receive placebo , riluzole 100 mg/day , or riluzole 200 mg/day .  The prespecified outcome measure was change in the total maximal chorea score of the Unified Huntington ’s Disease Rating Scale ( UHDRS ) . Results : Fifty-six ( 89 % ) subjects completed the study . A reduction ( p < 0.01 ) in chorea at 8 weeks was found using a linear trend test with dose . Comparing the groups individually , the reduction in chorea for the riluzole 200-mg/day group ( −2.2 ± 3.3 ) was different ( p = 0.01 ) from placebo ( + 0.7 ± 3.4 ) , but the riluzole 100-mg/day group ( −0.2 ± 2.9 ) was not . Riluzole did not improve other motor , cognitive , behavioral , or functional components of the UHDRS . Alanine aminotransferase was elevated in a dosage-dependent fashion ( p = 0.01 ) . Conclusions : Over 8 weeks of treatment , riluzole 200 mg/day ameliorated chorea intensity in HD without improving functional capacity or other clinical features of illness . Riluzole 200 mg/day was attended by reversible liver transaminase abnormalities that would require monitoring in long-term studies The objective of this study was to evaluate the effect of riluzole on dyskinesia and the duration of the ON state in patients with Parkinson disease ( PD ) . The authors studied 16 PD patients with levodopa-induced dyskinesia . All patients initially received an apomorphine dose intended to induce the motor function benefit ( ON state ) generally accompanied by dyskinesia . They evaluated the patients during the OFF and ON states using the UPDRS-III , UPDRS-IV , and Larsen scales , and measured the duration of the ON state . Patients were r and omly assigned to receive either riluzole ( 50 mg bid ) or placebo for 7 consecutive days ( 8 patients in each group ) . The authors did not interrupt previously prescribed medication . Following the 7-day period , they carried out similar evaluation procedures before and after another apomorphine challenge . Mean UPDRS-IV scores were 6.1 points and 6.0 points before and after riluzole therapy respectively . For the placebo group , the scores were 6.9 points and 6.6 points for the initial and final evaluations respectively . Larsen scale had mean scores of 9.2 points and 9.9 points for the pre- and postriluzole periods , and 10.2 points and 9.6 points for pre- and post-placebo evaluations respectively . The ON state was 33.5 % lengthier after 7 days of riluzole and 28.0 % lengthier after placebo . They could not find any statistical differences between the 2 groups . Short-term riluzole administration in PD patients was not able to reduce apomorphine-induced dyskinesia but could extend the ON state duration , although this did not reach statistical significance BACKGROUND To the extent that excitotoxicity may play a role in the pathogenesis of certain neurodegenerative disorders , antagonists of glutamate , an excitatory neurotransmitter , should exert neuroprotective effects in these disorders , including Parkinson 's disease ( PD ) . METHODS Patients in early stages of PD , not previously treated with levodopa , were r and omized to receive riluzole 50 mg capsules orally , taken twice daily or a matching placebo . All subjects were evaluated at baseline ( pre-treatment ) , at 1 , 3 and 6 months ( post-treatment ) , and following a 6-week washout . After the washout , all subjects were offered an enrollment in an open label , 1-year , extension study . The principal investigator ( JJ ) , however , remained blinded to the original assignment during the entire study . The patients were assessed by the Unified Parkinson 's Disease Rating Scale ( UPDRS ) , Activities of Daily Living ( ADL ) , Hoehn & Yahr ( HY ) stage , and Schwab and Engl and ( SE ) ADL scale . The quantitative assessment s included Movement Time ( MT ) and Reaction Time ( RT ) . Additionally , the time to initiate dopaminergic therapy  was assessed . Safety was determined at each visit by clinical history and examination , a panel of blood safety laboratory tests including complete blood count , chemistry profile , and liver function studies . RESULTS Twenty patients with a mean age of 62+/-9.02 ( range : 46 - 73 ) years and mean duration of symptoms of 18+/-9.53 ( range : 6 - 36 ) months were enrolled . One patient withdrew from the study because he needed more aggressive treatment of his symptoms . Analysis of the efficacy variables showed no meaningful symptomatic effect of riluzole on UPDRS score . Likewise , there was no significant change in the median HY stage , SE ADL rating , or the MT/RT . Seventeen patients ( mean age 62+/-9.26 ) elected to continue in the open label extension study . Although the observed deterioration in UPDRS scores seemed to be more pronounced in the placebo group than in the riluzole group , the difference did not reach statistical significance . There was no statistically significant difference in the latency between enrollment and start of symptomatic therapy when patients initially treated with riluzole were compared to those initially treated with placebo ( 8.3 vs 9 months ) . CONCLUSIONS This pilot and extension study showed that riluzole , 100mg/day , was well tolerated in patients with early PD . No evidence of symptomatic effect of riluzole was observed . Because of the exploratory nature of the design and small size of the study , it was not possible to determine whether riluzole affected the natural history of PD . The encouraging results from our study , however , suggest that larger , longitudinal studies are warranted BACKGROUND Our previous study in patients with cerebellar ataxias of different causes showed significant benefit of riluzole after 8 weeks . We aim ed to confirm these results in patients with spinocerebellar ataxia or Friedreich 's ataxia in a 1-year trial . METHODS Patients with spinocerebellar ataxia or Friedreich 's ataxia ( 2:1 ratio ) from three Italian neurogenetic units were enrolled in this multicentre , double-blind , placebo-controlled trial , and r and omly assigned to riluzole ( 50 mg orally , twice daily ) or placebo for 12 months . The r and omisation list was computer-generated and a central ised r and omisation system was implemented . Participants and assessing neurologists were masked to treatment allocation . The primary endpoint was the proportion of patients with improved Scale for the Assessment and Rating of Ataxia ( SARA ) score ( a drop of at least one point ) at 12 months . An intention-to-treat analysis was done . This trial is registered at Clinical Trials.gov , number NCT01104649 . FINDINGS Between May 22 , 2010 , and Feb 25 , 2013 , 60 patients were enrolled . Two patients in the riluzole group and three in the placebo group withdrew their consent before receiving treatment , so the intention-to-treat analysis was done on 55 patients ( 19 with spinocerebellar ataxia and nine with Friedreich 's ataxia in the riluzole group , and 19 with spinocerebellar ataxia and eight with Fried 
    Output:
    
No significant difference was found in the number of participants with adverse events but with motor improvement in hereditary ataxia .
Riluzole is well-tolerated in the patients with neurodegenerative movement disorders .
Moreover , it makes sense to design long-term study focusing on neuroprotective effect of riluzole in disease-modifying