International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome

Trouillas, P; Takayanagi, Tetsuya; Hallett, Mark; Currier, Robert D.; Subramony, S. H.; Wessel, K.; Bryer, Alan; Diener, Hans Christoph; Massaquoi, Steve G.; Gómez, Christopher M.; Coutinho, Paula; Hamida, M. Ben; Campanella, G; Filla, Alessandro; Schut, Lawrence J.; Timann, D.; Honnorat, J; Nighoghossian, Norbert; Manyam, Bala V.

doi:10.1016/s0022-510x(96)00231-6

Cited by 1,140 publications

(787 citation statements)

References 26 publications

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“…At enrollment (T 0 ), 16‐week follow‐up (T 1 ), and after 40‐week DHA supplementation (T 2 ), each patient underwent standardized clinical assessment (ie, Scale for the Assessment and Rating of Ataxia [SARA]8 and the International Cooperative Ataxia Rating Scale [ICARS]9, brain 18‐fluorodeoxyglucose [FDG] positron emission tomography [PET] scan, electromyography [EMG]/electroneurography [ENG], and blood sampling for biological analyses).…”

Section: Methodsmentioning

confidence: 99%

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Docosahexaenoic acid is a beneficial replacement treatment for spinocerebellar ataxia 38

Manes

Alberici

Gregorio

et al. 2017

Annals of Neurology

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ObjectiveSpinocerebellar ataxia 38 (SCA38) is caused by mutations in the ELOVL5 gene, which encodes an elongase involved in the synthesis of polyunsaturated fatty acids, including docosahexaenoic acid (DHA). As a consequence, DHA is significantly reduced in the serum of SCA38 subjects. In the present study, we evaluated the safety of DHA supplementation, its efficacy for clinical symptoms, and changes of brain functional imaging in SCA38 patients.MethodsWe enrolled 10 SCA38 patients, and carried out a double‐blind randomized placebo‐controlled study for 16 weeks, followed by an open‐label study with overall 40‐week DHA treatment. At baseline and at follow‐up visit, patients underwent standardized clinical assessment, brain 18‐fluorodeoxyglucose positron emission tomography, electroneurography, and ELOVL5 expression analysis.ResultsAfter 16 weeks, we showed a significant pre–post clinical improvement in the DHA group versus placebo, using the Scale for the Assessment and Rating of Ataxia (SARA; mean difference [MD] = +2.70, 95% confidence interval [CI] = +0.13 to + 5.27, p = 0.042). At 40‐week treatment, clinical improvement was found significant by both SARA (MD = +2.2, 95% CI = +0.93 to + 3.46, p = 0.008) and International Cooperative Ataxia Rating Scale (MD = +3.8, 95% CI = +1.39 to + 6.41, p = 0.02) scores; clinical data were corroborated by significant improvement of cerebellar hypometabolism (statistical parametric mapping analyses, false discovery rate corrected). We also showed a decreased expression of ELOVL5 in patients’ blood at 40 weeks as compared to baseline. No side effect was recorded.InterpretationDHA supplementation is a safe and effective treatment for SCA38, showing an improvement of clinical symptoms and cerebellar hypometabolism. Ann Neurol 2017;82:615–621

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Section: Methodsmentioning

confidence: 99%

“…At each time point, videotapes of the SARA (range = 0–40)8 and the ICARS (range = 0–100)9 were employed to evaluate cerebellar deficits. Intra‐assay variability of SARA and ICARS scores was evaluated between 2 independent neurologists (A.A. and M.M., SARA and ICARS alpha‐Cronbach = 0.983 and 0.995, respectively).…”

Section: Methodsmentioning

confidence: 99%

Docosahexaenoic acid is a beneficial replacement treatment for spinocerebellar ataxia 38

Manes

Alberici

Gregorio

et al. 2017

Annals of Neurology

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“…For evaluation, we adopted the SCDscore, established for clinical evaluations of the symptoms of SCDpatients based on the ataxia evaluating manual of TRH-Tin a multi-center, placebo-controlled double blind study (1). The International Cooperative Ataxia Rating Scale, which the Ataxia Neuropharmacology Committee of the World Federation of Neurology proposed for the pharmacological assessment of cerebellar ataxia, was not yet available at the commencement of this trial (12). In our trial, patients whoshowed improvementwere at the mild or moderate stages.…”

Section: Discussionmentioning

confidence: 99%

Branched-Chain Amino Acid Therapy for Spinocerebellar Degeneration: A Pilot Clinical Crossover Trial.

et al. 1999

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Object: The potential effects of branched-chain amino acids (BCAAs)on spinocerebellar degeneration (SCD) were explored in eleven patients. Methods: The patients received 200 ml of BCAA-rich solution, 2 mg of thyrotropin-releasing hormone (TRH; protirelin), or a placebo daily for 7 days each in a random order. An SCDscore was used to quantify the severity of symptoms. Patients: Eleven patients with SCD(7 male, 4 female; mean age 60±ll; mean disease duration 5.5 years) participated in this study. Results: The meanSCDscore of the eleven patients improved significantly by the BCAAtreatment compared with the baseline. The conditions of five of the eleven patients (45 %) were clearly improved by the BCAAtreatment. All of the responders manifested predominantly cerebellar symptoms, but no prominent parkinsonian symptoms. Two patients with marked rigidity and akinesia did not respond to the treatment. Conclusion: Weconcluded that BCAAsdo have a beneficial effect on functional improvement in patients with SCD, and that further large scale studies are needed. (Internal Medicine 38: 401-406, 1999)

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“…Fundoscopy showed pigmentary retinopathy (Fig 1). To score the cerebellar dysfunction, we used the International Cooperative Ataxia Rating Scale (ICARS) 3 . The patient obtained a 49 score.…”

Section: Casementioning

confidence: 99%

Hypergonadotropic hypogonadism and cerebellar ataxia: an unusual association

Braga‐Neto

Santos-Neto

et al. 2010

Arq. Neuro-Psiquiatr.

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International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome

Cited by 1,140 publications

References 26 publications

Docosahexaenoic acid is a beneficial replacement treatment for spinocerebellar ataxia 38

Docosahexaenoic acid is a beneficial replacement treatment for spinocerebellar ataxia 38

Branched-Chain Amino Acid Therapy for Spinocerebellar Degeneration: A Pilot Clinical Crossover Trial.

Hypergonadotropic hypogonadism and cerebellar ataxia: an unusual association

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