Journal article
Defining disability: development and validation of a mobility-Disability Severity Index (mDSI) in Charcot-Marie-tooth disease
Journal of neurology, neurosurgery and psychiatry, Vol.86(6), pp.635-639
06/2015
DOI: 10.1136/jnnp-2013-307390
PMCID: PMC4920058
PMID: 25157034
Abstract
Objective To develop and validate a reliable patient-reported scale that grades the severity of disability in Charcot-Marie-tooth disease (CMT), from an in-depth analysis of patient and healthcare provider perspectives on what mobility changes constitutes mild, moderate and severe disability. Design In this prospective, cross-sectional study, a 19-item Disability Questionnaire was developed following literature and expert review. Between 2011 and 2012, the Disability Questionnaire was provided to healthcare providers experienced in CMT attending national scientific meetings, and to patients self-registered with the Inherited Neuropathy Consortium—Rare Diseases Clinical Research Consortium on-line contact registry. Provider and patient responses were compared utilising a two-sided unpaired t test with Bonferroni correction. The questionnaire was then assessed for validity, reliability and unidimensionality. Results We analysed 259 Disability Questionnaires (167 patients, 92 providers); these showed perfect agreement between patient and provider responses on qualitative descriptions of disability, but significant differences in quantitative responses on items corresponding to minimal or severe disability (p<0.001). Validity and test–retest reliability of the questionnaire was excellent (Cronbach's α=0.96; intraclass correlation coefficients (ICC)=0.977 (0.951 to 0.993). Exploratory factor analysis and the Mokken Scaling Procedure supported the unidimensionality of the mobility-Disability Severity Index. Conclusions The mobility-Disability Severity Index is a unique instrument, categorising disability from the patient's perspective, and will undergo further cross-validation studies in CMT.
Details
- Title: Subtitle
- Defining disability: development and validation of a mobility-Disability Severity Index (mDSI) in Charcot-Marie-tooth disease
- Creators
- Sindhu Ramchandren - Department of Neurology, University of Michigan, Ann Arbor, Michigan, USAMichael Shy - Department of Neurology, University of Iowa, Iowa City, Iowa, USAEva Feldman - Department of Neurology, University of Michigan, Ann Arbor, Michigan, USARuth Carlos - Department of Radiology, University of Michigan, Ann Arbor, Michigan, USACarly Siskind - Department of Neurology, Stanford Hospital and Clinics, Stanford, California, USA
- Resource Type
- Journal article
- Publication Details
- Journal of neurology, neurosurgery and psychiatry, Vol.86(6), pp.635-639
- DOI
- 10.1136/jnnp-2013-307390
- PMID
- 25157034
- PMCID
- PMC4920058
- NLM abbreviation
- J Neurol Neurosurg Psychiatry
- ISSN
- 0022-3050
- eISSN
- 1468-330X
- Grant note
- This study was supported by the NIH: K23-NS072279- Dr. Ramchandren; NINDS/ORD U54-NS065712, the Charcot Marie Tooth Association, and the Muscular Dystrophy Association- Dr. Shy; and The A. Alfred Taubman Medical Research Institute- Dr. Feldman. No funding organization or sponsor had any role in the design and conduct of the study; collection, management, analysis and interpretation of the data; and preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.
- Comment
- Test development: Disability Questionnaire (mobility-Disability Severity Index)
- Language
- English
- Date published
- 06/2015
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Stead Family Department of Pediatrics; Iowa Neuroscience Institute
- Record Identifier
- 9984020605302771
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