Literature DB >> 16769946

Scale for the assessment and rating of ataxia: development of a new clinical scale.

T Schmitz-Hübsch1, S Tezenas du Montcel, L Baliko, J Berciano, S Boesch, C Depondt, P Giunti, C Globas, J Infante, J-S Kang, B Kremer, C Mariotti, B Melegh, M Pandolfo, M Rakowicz, P Ribai, R Rola, L Schöls, S Szymanski, B P van de Warrenburg, A Dürr, T Klockgether, Roberto Fancellu.   

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.

Entities:  

Mesh:

Year:  2006        PMID: 16769946     DOI: 10.1212/01.wnl.0000219042.60538.92

Source DB:  PubMed          Journal:  Neurology        ISSN: 0028-3878            Impact factor:   9.910


  535 in total

1.  Linear path integration deficits in patients with abnormal vestibular afference.

Authors:  Joeanna C Arthur; Kathleen B Kortte; Mark Shelhamer; Michael C Schubert
Journal:  Seeing Perceiving       Date:  2012

2.  Cognitive deficits in Machado-Joseph disease correlate with hypoperfusion of visual system areas.

Authors:  Pedro Braga-Neto; Lívia Almeida Dutra; José Luiz Pedroso; André C Felício; Helena Alessi; Ruth F Santos-Galduroz; Paulo Henrique F Bertolucci; Mário Luiz V Castiglioni; Rodrigo Affonseca Bressan; Griselda Esther Jara de Garrido; Orlando Graziani Povoas Barsottini; Andrea Jackowski
Journal:  Cerebellum       Date:  2012-12       Impact factor: 3.847

3.  Exome sequencing reveals a homozygous SYT14 mutation in adult-onset, autosomal-recessive spinocerebellar ataxia with psychomotor retardation.

Authors:  Hiroshi Doi; Kunihiro Yoshida; Takao Yasuda; Mitsunori Fukuda; Yoko Fukuda; Hiroshi Morita; Shu-ichi Ikeda; Rumiko Kato; Yoshinori Tsurusaki; Noriko Miyake; Hirotomo Saitsu; Haruya Sakai; Satoko Miyatake; Masaaki Shiina; Nobuyuki Nukina; Shigeru Koyano; Shoji Tsuji; Yoshiyuki Kuroiwa; Naomichi Matsumoto
Journal:  Am J Hum Genet       Date:  2011-08-12       Impact factor: 11.025

4.  The natural history of spinocerebellar ataxia type 1, 2, 3, and 6: a 2-year follow-up study.

Authors:  H Jacobi; P Bauer; P Giunti; R Labrum; M G Sweeney; P Charles; A Dürr; C Marelli; C Globas; C Linnemann; L Schöls; M Rakowicz; R Rola; E Zdzienicka; T Schmitz-Hübsch; R Fancellu; C Mariotti; C Tomasello; L Baliko; B Melegh; A Filla; C Rinaldi; B P van de Warrenburg; C C P Verstappen; S Szymanski; J Berciano; J Infante; D Timmann; S Boesch; S Hering; C Depondt; M Pandolfo; J-S Kang; S Ratzka; J Schulz; S Tezenas du Montcel; T Klockgether
Journal:  Neurology       Date:  2011-08-10       Impact factor: 9.910

5.  Diffusion tensor imaging of spinocerebellar ataxias types 1 and 2.

Authors:  M L Mandelli; T De Simone; L Minati; M G Bruzzone; C Mariotti; R Fancellu; M Savoiardo; M Grisoli
Journal:  AJNR Am J Neuroradiol       Date:  2007 Nov-Dec       Impact factor: 3.825

6.  Inventory of Non-Ataxia Signs (INAS): validation of a new clinical assessment instrument.

Authors:  H Jacobi; M Rakowicz; R Rola; R Fancellu; C Mariotti; P Charles; A Dürr; M Küper; D Timmann; C Linnemann; L Schöls; O Kaut; C Schaub; A Filla; L Baliko; B Melegh; J-S Kang; P Giunti; B P C van de Warrenburg; R Fimmers; T Klockgether
Journal:  Cerebellum       Date:  2013-06       Impact factor: 3.847

7.  Follow-up of pediatric patients treated by IVIG for Langerhans cell histiocytosis (LCH)-related neurodegenerative CNS disease.

Authors:  Shinsaku Imashuku; Naoto Fujita; Yoko Shioda; Haruyoshi Noma; Shiro Seto; Toshinori Minato; Kazuo Sakashita; Nobuhiro Ito; Ryoji Kobayashi; Akira Morimoto
Journal:  Int J Hematol       Date:  2014-12-10       Impact factor: 2.490

Review 8.  A severity scoring tool to assess the neurological features of neuronopathic Gaucher disease.

Authors:  E H Davies; R Surtees; C DeVile; I Schoon; A Vellodi
Journal:  J Inherit Metab Dis       Date:  2007-09-16       Impact factor: 4.982

9.  Paraneoplastic cerebellar ataxia associated with anti-Hu antibodies and benign ganglioneuroma.

Authors:  Roberto Fancellu; Elena Corsini; Giorgio Bernardi; Paolo Buzzo; Maria Luisa Ferrari; Eleonora Lamantea; Alberto Garaventa; Mauro Truini; Sandro Salvarani
Journal:  Funct Neurol       Date:  2014 Oct-Dec

10.  Clinical and Genetic Evaluation of Spinocerebellar Ataxia Type 10 in 16 Brazilian Families.

Authors:  Bernardo Machado Dias Domingues; Fábio A Nascimento; Alex Tiburtino Meira; Adriana Moro; Salmo Raskin; Tetsuo Ashizawa; Hélio Afonso Ghizoni Teive
Journal:  Cerebellum       Date:  2019-10       Impact factor: 3.847

View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.