Reliability of phenotypic early-onset ataxia assessment : A pilot study
(2016) In Developmental Medicine and Child Neurology 58(1). p.70-76- Abstract
Aim: To investigate the interobserver agreement on phenotypic early-onset ataxia (EOA) assessment and to explore whether the Scale for Assessment and Rating of Ataxia (SARA) could provide a supportive marker. Method: Seven movement disorder specialists provided independent phenotypic assessments of potentially ataxic motor behaviour in 40 patients (mean age 15y [range 5-34]; data derived from University Medical Center Groningen medical records 1998-2012). We determined interobserver agreement by Fleiss' kappa. Furthermore, we compared percentage SARA subscores ([subscore/total score]×100%) between 'indisputable' (primary ataxia recognition by at least six observers) and 'mixed' (ataxia recognition, unfulfilling 'indisputable' criteria)... (More)
Aim: To investigate the interobserver agreement on phenotypic early-onset ataxia (EOA) assessment and to explore whether the Scale for Assessment and Rating of Ataxia (SARA) could provide a supportive marker. Method: Seven movement disorder specialists provided independent phenotypic assessments of potentially ataxic motor behaviour in 40 patients (mean age 15y [range 5-34]; data derived from University Medical Center Groningen medical records 1998-2012). We determined interobserver agreement by Fleiss' kappa. Furthermore, we compared percentage SARA subscores ([subscore/total score]×100%) between 'indisputable' (primary ataxia recognition by at least six observers) and 'mixed' (ataxia recognition, unfulfilling 'indisputable' criteria) EOA phenotypes. Results: Agreement on phenotypic EOA assessment was statistically significant (p<0.001), but of moderate strength (Fleiss' kappa=0.45; 95% CI 0.38-0.51). During mild disease progression, percentage SARA gait subscores discriminated between 'indisputable' and 'mixed' EOA phenotypes. In patients with percentage SARA gait subscores >30%, primary ataxia was more frequently present than in those with subscores <30% (p=0.001). Interpretation: Among movement-disorder professionals from different disciplines, interobserver agreement on phenotypic EOA recognition is of limited strength. SARA gait subscores can provide a supportive discriminative marker between EOA phenotypes. Hopefully, future phenotypic insight will contribute to the inclusion of uniform, high-quality data in international EOA databases.
(Less)
- author
- Lawerman, Tjitske F. ; Brandsma, Rick ; van Geffen, Joke T. ; Lunsing, Roelineke J. ; Burger, Huibert ; Tijssen, Marina A.J. ; de Vries, Jeroen J. ; de Koning, Tom J. LU and Sival, Deborah A.
- publishing date
- 2016-01-01
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Developmental Medicine and Child Neurology
- volume
- 58
- issue
- 1
- pages
- 7 pages
- publisher
- John Wiley & Sons Inc.
- external identifiers
-
- scopus:84955193704
- pmid:25995073
- ISSN
- 0012-1622
- DOI
- 10.1111/dmcn.12804
- language
- English
- LU publication?
- no
- id
- 60c64fe7-5eb9-47d2-9a5e-9399af41beb3
- date added to LUP
- 2020-02-26 09:58:49
- date last changed
- 2024-10-02 22:34:31
@article{60c64fe7-5eb9-47d2-9a5e-9399af41beb3, abstract = {{<p>Aim: To investigate the interobserver agreement on phenotypic early-onset ataxia (EOA) assessment and to explore whether the Scale for Assessment and Rating of Ataxia (SARA) could provide a supportive marker. Method: Seven movement disorder specialists provided independent phenotypic assessments of potentially ataxic motor behaviour in 40 patients (mean age 15y [range 5-34]; data derived from University Medical Center Groningen medical records 1998-2012). We determined interobserver agreement by Fleiss' kappa. Furthermore, we compared percentage SARA subscores ([subscore/total score]×100%) between 'indisputable' (primary ataxia recognition by at least six observers) and 'mixed' (ataxia recognition, unfulfilling 'indisputable' criteria) EOA phenotypes. Results: Agreement on phenotypic EOA assessment was statistically significant (p<0.001), but of moderate strength (Fleiss' kappa=0.45; 95% CI 0.38-0.51). During mild disease progression, percentage SARA gait subscores discriminated between 'indisputable' and 'mixed' EOA phenotypes. In patients with percentage SARA gait subscores >30%, primary ataxia was more frequently present than in those with subscores <30% (p=0.001). Interpretation: Among movement-disorder professionals from different disciplines, interobserver agreement on phenotypic EOA recognition is of limited strength. SARA gait subscores can provide a supportive discriminative marker between EOA phenotypes. Hopefully, future phenotypic insight will contribute to the inclusion of uniform, high-quality data in international EOA databases.</p>}}, author = {{Lawerman, Tjitske F. and Brandsma, Rick and van Geffen, Joke T. and Lunsing, Roelineke J. and Burger, Huibert and Tijssen, Marina A.J. and de Vries, Jeroen J. and de Koning, Tom J. and Sival, Deborah A.}}, issn = {{0012-1622}}, language = {{eng}}, month = {{01}}, number = {{1}}, pages = {{70--76}}, publisher = {{John Wiley & Sons Inc.}}, series = {{Developmental Medicine and Child Neurology}}, title = {{Reliability of phenotypic early-onset ataxia assessment : A pilot study}}, url = {{http://dx.doi.org/10.1111/dmcn.12804}}, doi = {{10.1111/dmcn.12804}}, volume = {{58}}, year = {{2016}}, }