Journal ArticleDOI
Scale for the assessment and rating of ataxia: development of a new clinical scale.
T. Schmitz-Hübsch,S. Tezenas du Montcel,Laszlo Baliko,José Berciano,S Boesch,Chantal Depondt,Paola Giunti,C. Globas,Jon Infante,J-S Kang,Berry Kremer,Christian Mariotti,Béla Melegh,Massimo Pandolfo,Maryla Rakowicz,P Ribai,Rafał Rola,Ludger Schöls,Sandra Szymanski,B.P.C. van de Warrenburg,Alexandra Durr,Thomas Klockgether,Roberto Fancellu +22 more
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TLDR
The Scale for the Assessment and Rating of Ataxia is a reliable and valid measure of ataxia, making it an appropriate primary outcome measure for clinical trials.Abstract:
OBJECTIVE: To develop a reliable and valid clinical scale measuring the severity of ataxia. METHODS: The authors devised the Scale for the Assessment and Rating of Ataxia (SARA) and tested it in two trials of 167 and 119 patients with spinocerebellar ataxia. RESULTS: The mean time to administer SARA in patients was 14.2 +/- 7.5 minutes (range 5 to 40). Interrater reliability was high, with an intraclass coefficient (ICC) of 0.98. Test-retest reliability was high with an ICC of 0.90. Internal consistency was high as indicated by Cronbach's alpha of 0.94. Factorial analysis revealed that the rating results were determined by a single factor. SARA ratings showed a linear relation to global assessments using a visual analogue scale, suggesting linearity of the scale (p < 0.0001, r(2) = 0.98). SARA score increased with the disease stage (p < 0.001) and was closely correlated with the Barthel Index (r = -0.80, p < 0.001) and part IV (functional assessment) of the Unified Huntington's Disease Rating Scale (UHDRS-IV) (r = -0.89, p < 0.0001), whereas it had only a weak correlation with disease duration (r = 0.34, p < 0.0002). CONCLUSIONS: The Scale for the Assessment and Rating of Ataxia is a reliable and valid measure of ataxia, making it an appropriate primary outcome measure for clinical trials.read more
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Journal ArticleDOI
Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7.
Udo Rüb,Ludger Schöls,Henry L. Paulson,Georg Auburger,Pawel Kermer,Joanna C. Jen,Kay Seidel,Horst-Werner Korf,Thomas Deller +8 more
TL;DR: The present review summarizes the current knowledge about the polyglutamine ataxias SCA1,SCA2, SCA3, SCa6 and SCA7 and compares their clinical and electrophysiological features, genetic and molecular biological background, as well as their brain pathologies.
Journal ArticleDOI
Consensus Paper: Revisiting the Symptoms and Signs of Cerebellar Syndrome
Florian Bodranghien,Amy J. Bastian,Carlo Casali,Mark Hallett,Elan D. Louis,Mario Manto,Peter Mariën,D. A. Nowak,Jeremy D. Schmahmann,Mariano Serrao,Katharina M Steiner,Michael Strupp,Caroline Tilikete,Dagmar Timmann,Kim van Dun +14 more
TL;DR: The concept of the cerebellar syndrome is revisited in the light of recent advances in the understanding of Cerebellar operations and the key symptoms and signs of cerebellars dysfunction are discussed.
Journal ArticleDOI
Spinocerebellar ataxia types 1, 2, 3, and 6 Disease severity and nonataxia symptoms
T. Schmitz-Hübsch,M Coudert,Peter Bauer,Paola Giunti,C. Globas,Laszlo Baliko,Alessandro Filla,Christian Mariotti,Maryla Rakowicz,Perrine Charles,P Ribai,P Ribai,Sandra Szymanski,Jon Infante,B.P.C. van de Warrenburg,Alexandra Durr,Alexandra Durr,Dagmar Timmann,S Boesch,Roberto Fancellu,Rafał Rola,Chantal Depondt,Ludger Schöls,E Zdienicka,J-S Kang,S Döhlinger,Berry Kremer,D A Stephenson,Béla Melegh,Massimo Pandolfo,S. Di Donato,S. Tezenas du Montcel,Thomas Klockgether +32 more
TL;DR: This study suggests that spinocerebellar ataxia (SCA) 1, SCA2, and SCA3 share a number of common biologic properties, whereas SCA6 is distinct in that its phenotype is more determined by age than by disease-related factors.
Journal ArticleDOI
Intensive coordinative training improves motor performance in degenerative cerebellar disease.
TL;DR: In this paper, the effectiveness of a 4-week intensive coordinative training for 16 patients with progressive ataxia due to cerebellar degeneration or degeneration of afferent pathways was examined.
Journal ArticleDOI
Diagnosis and treatment of Friedreich ataxia: a European perspective
Jörg B. Schulz,Sylvia Boesch,Katrin Bürk,Alexandra Durr,Paola Giunti,Caterina Mariotti,Françoise Pousset,Ludger Schöls,Pierre Vankan,Massimo Pandolfo +9 more
TL;DR: This Review provides guidelines, from a European perspective, for the diagnosis of Friedreich ataxia, differential diagnosis of ataxias and genetic counseling, and treatment of neurological and non-neurological symptoms.
References
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Journal ArticleDOI
Unified huntington’s disease rating scale: Reliability and consistency
Karl Kieburtz,John B. Penney,Peter Corno,Neal G. Ranen,Ira Shoulson,Andrew Feigin,Davi Abwender,J. Timothy Greenarnyre,Donald S. Higgins,Frederick J. Marshall,Joshua L. Goldstein,Kimberly Steinberg,Charles Shih,Irene H. Richard,Charlyne Hickey,Carol Zimmerman,Constance Orme,Kathy Claude,David Oakes,Daniel S. Sax,Anthony Kim,Steven M. Hersch,Randi Jones,Alexander P. Auchus,David B. Olsen,Cheryl Bissey-Black,Allen Rubin,Rose Schwartz,Richard Dubinsky,William Mallonee,Carolyn Gray,Nan Godfrey,Greg Suter,Kathleen M. Shannon,Glenn T. Stebbins,Jean A. Jaglin,Karen Marder,Stuart Taylor,Elan D. Louis,Carol Moskowitz,Deborah Zeck Thorne,Naomi Zubin,Nancy S. Wexler,Michael R. Swenson,Jane S. Paulsen,Neal R. Swerdlow,Roger L. Albin,Christine Wernette,Francis O. Walker,Vicki Hunt +49 more
TL;DR: The limited longitudinal database indicates that the UHDRS may be useful for tracking changes in the clinical features of HD over time and there was an excellent degree of interrater reliability for the motor scores.
Journal Article
Unified Huntington's disease rating scale: reliability and consistency
TL;DR: The limited longitudinal database indicates that the UHDRS may be useful for tracking changes in the clinical features of HD over time and there was an excellent degree of interrater reliability for the motor scores.
Journal ArticleDOI
International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome
Paul Trouillas,Tetsuya Takayanagi,Mark Hallett,Robert D. Currier,S. H. Subramony,K. Wessel,Alan Bryer,Hans-Christoph Diener,S. Massaquoi,Christopher M. Gomez,Paula Coutinho,M. Ben Hamida,Giuseppe Campanella,Alessandro Filla,L. Schut,D. Timann,Jérôme Honnorat,Norbert Nighoghossian,Bala V. Manyam +18 more
TL;DR: The scale proposed involves a compartimentalized quantification of postural and stance disorders, limb ataxia, dysarthria and oculomotor disorders, in order that a subscore concerning these symptoms may be separately studied.
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International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome
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Spinocerebellar ataxia types 1, 2, 3, and 6 Disease severity and nonataxia symptoms
T. Schmitz-Hübsch,M Coudert,Peter Bauer,Paola Giunti,C. Globas,Laszlo Baliko,Alessandro Filla,Christian Mariotti,Maryla Rakowicz,Perrine Charles,P Ribai,P Ribai,Sandra Szymanski,Jon Infante,B.P.C. van de Warrenburg,Alexandra Durr,Alexandra Durr,Dagmar Timmann,S Boesch,Roberto Fancellu,Rafał Rola,Chantal Depondt,Ludger Schöls,E Zdienicka,J-S Kang,S Döhlinger,Berry Kremer,D A Stephenson,Béla Melegh,Massimo Pandolfo,S. Di Donato,S. Tezenas du Montcel,Thomas Klockgether +32 more