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Reliability of phenotypic early-onset ataxia assessment : A pilot study

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. (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.

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author
; ; ; ; ; ; ; and
publishing date
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
  • pmid:25995073
  • scopus:84955193704
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
2024-01-02 06:27:13
@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&lt;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 &gt;30%, primary ataxia was more frequently present than in those with subscores &lt;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}},
}