TY - JOUR
T1 - Quantifiable evaluation of cerebellar signs in children
AU - Filipovic Pierucci, Antoine
AU - Mariotti, Caterina
AU - Panzeri, Marta
AU - Giunti, Paola
AU - Boesch, Sylvia
AU - Schulz, Jörg B.
AU - Pandolfo, Massimo
AU - Durr, Alexandra
AU - Tezenas Du Montcel, Sophie
PY - 2015/3/24
Y1 - 2015/3/24
N2 - 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.
AB - 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.
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U2 - 10.1212/WNL.0000000000001403
DO - 10.1212/WNL.0000000000001403
M3 - Article
C2 - 25716360
AN - SCOPUS:84925427566
VL - 84
SP - 1225
EP - 1232
JO - Neurology
JF - Neurology
SN - 0028-3878
IS - 12
ER -