This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
Abstract
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.
GLOSSARY
- CCFS=
- composite cerebellar functional severity;
- EFACTS=
- European Friedreich's Ataxia Consortium for Translational Studies;
- FRDA=
- Friedreich ataxia;
- SARA=
- Scale for the Assessment and Rating of Ataxias;
- SCA=
- spinocerebellar ataxia
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Coinvestigators from the EFACTS Study Group are listed on the Neurology® Web site at Neurology.org.
Supplemental data at Neurology.org
- Received September 17, 2014.
- Accepted in final form December 1, 2014.
- © 2015 American Academy of Neurology
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
