Reliability and discriminant validity of ataxia rating scales in early onset ataxia

To determine whether ataxia rating scales are reliable disease biomarkers for early onset ataxia (EOA).

[1]  M. Tijssen,et al.  Reliability of phenotypic early‐onset ataxia assessment: a pilot study , 2016, Developmental medicine and child neurology.

[2]  R. Artuch,et al.  Idebenone treatment in paediatric and adult patients with Friedreich ataxia: long-term follow-up. , 2008, European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society.

[3]  H. Kremer,et al.  Riluzole in patients with hereditary cerebellar ataxia , 2016, The Lancet Neurology.

[4]  H. Sasaki,et al.  Usefulness of the Scale for Assessment and Rating of Ataxia (SARA) , 2008, Journal of the Neurological Sciences.

[5]  A. Stray-Pedersen,et al.  Prevalence of hereditary ataxia and spastic paraplegia in southeast Norway: a population-based study. , 2009, Brain : a journal of neurology.

[6]  Paola Giunti,et al.  Reliability and validity of the International Cooperative Ataxia Rating Scale: A study in 156 spinocerebellar ataxia patients , 2006, Movement disorders : official journal of the Movement Disorder Society.

[7]  Markus Frings,et al.  Reliability and validity of the scale for the assessment and rating of ataxia: A study in 64 ataxia patients , 2007, Movement disorders : official journal of the Movement Disorder Society.

[8]  M. Pranzatelli Movement disorders in childhood. , 1996, Pediatrics in review.

[9]  Application of a Scale for the Assessment and Rating of Ataxia (SARA) in Friedreich's ataxia patients according to posturography is limited , 2014, Journal of the Neurological Sciences.

[10]  F. Orzi,et al.  Riluzole in patients with hereditary cerebellar ataxia: a randomised, double-blind, placebo-controlled trial , 2015, The Lancet Neurology.

[11]  T Klockgether,et al.  The natural history of degenerative ataxia: a retrospective study in 466 patients. , 1998, Brain : a journal of neurology.

[12]  N. Maurits,et al.  In children with Friedreich ataxia, muscle and ataxia parameters are associated , 2011, Developmental medicine and child neurology.

[13]  M. Delgado,et al.  Movement Disorders in Children: Definitions, Classifications, and Grading Systems , 2003, Journal of child neurology.

[14]  N. Maurits,et al.  Neurophysiological evaluation in children with Friedreich's ataxia. , 2009, Early human development.

[15]  A. Durr Rare inherited diseases merit disease-specific trials , 2015, The Lancet Neurology.

[16]  M. Hallett,et al.  International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome , 1997, Journal of the Neurological Sciences.

[17]  Lippincott Williams Wilkins,et al.  Scale for the assessment and rating of ataxia: Development of a new clinical scale , 2006, Neurology.

[18]  D. Sival,et al.  The International Cooperative Ataxia Rating Scale shows strong age‐dependency in children , 2009, Developmental medicine and child neurology.

[19]  Kevin A Hallgren,et al.  Computing Inter-Rater Reliability for Observational Data: An Overview and Tutorial. , 2012, Tutorials in quantitative methods for psychology.

[20]  Jeremy D. Schmahmann,et al.  Development of a brief ataxia rating scale (BARS) based on a modified form of the ICARS , 2009, Movement disorders : official journal of the Movement Disorder Society.

[21]  H. Kremer,et al.  Ataxia rating scales are age‐dependent in healthy children , 2014, Developmental medicine and child neurology.

[22]  J. R. Landis,et al.  The measurement of observer agreement for categorical data. , 1977, Biometrics.

[23]  D. Cicchetti Guidelines, Criteria, and Rules of Thumb for Evaluating Normed and Standardized Assessment Instruments in Psychology. , 1994 .

[24]  L. Orsi,et al.  Early onset cerebellar ataxia with retained tendon reflexes: prevalence and gene frequency in an Italian population , 1993, Clinical genetics.