Quantifiable evaluation of cerebellar signs in children

Antoine Filipovic Pierucci, Caterina Mariotti, Marta Panzeri, Paola Giunti, Sylvia Boesch, Jörg B. Schulz, Massimo Pandolfo, Alexandra Durr, Sophie Tezenas Du Montcel

Research output: Contribution to journalArticlepeer-review


Objective: To validate, examine the internal validity, and adapt to children the electronic version of the composite cerebellar functional severity (CCFS) score. Methods: In this multicenter study, we compared the validated manual device with the new electronic version (n 46) and analyzed its kinetics in 146 patients with Friedreich ataxia through the EFACTS (European Friedreich's Ataxia Consortium for Translational Studies) network, 77 patients with spinocerebellar ataxia, and 48 controls. We validated the CCFS in cerebellar ataxias in healthy children (n 120) and children with Friedreich ataxia through the EFACTS network (n 33). Results: We showed that the electronic CCFS is a reliable replacement for the manual version (intraclass correlation coefficient: 0.98 [0.97-0.99]), and that the electronic CCFS is consistent when performed several times (0.92 [0.84-0.97]). Analysis of kinetics data showed an acceleration and irregularity that is not relevant compared with total speed. The CCFS was tested after modification in a population of patients with Friedreich ataxia between 8 and 19 years old, and showed similar values as adult patients with Friedreich ataxia (1.203 ± 0.125 vs 1.228 ± 0.167) and significantly higher values than controls of the same age (0.863 ± 0.042). Conclusions: The electronic CCFS is a quantified measurement of cerebellar ataxia independent of age, usable in individuals aged from 7 to 80 years. The automated nature of the electronic test device makes it reproducible between operators and centers, as well as easy to use.

Original languageEnglish
Pages (from-to)1225-1232
Number of pages8
Issue number12
Publication statusPublished - Mar 24 2015

ASJC Scopus subject areas

  • Clinical Neurology
  • Medicine(all)


Dive into the research topics of 'Quantifiable evaluation of cerebellar signs in children'. Together they form a unique fingerprint.

Cite this