Reliability of the CMT neuropathy score (second version) in Charcot-Marie-Tooth disease

Sinéad M Murphy; David N Herrmann; Michael P McDermott; Steven S Scherer; Michael E Shy; Mary M Reilly; Davide Pareyson

doi:10.1111/j.1529-8027.2011.00350.x

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Reliability of the CMT neuropathy score (second version) in Charcot-Marie-Tooth disease

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Reliability of the CMT neuropathy score (second version) in Charcot-Marie-Tooth disease

Sinéad M Murphy, David N Herrmann, Michael P McDermott, Steven S Scherer, Michael E Shy, Mary M Reilly and Davide Pareyson

Journal of the peripheral nervous system, Vol.16(3), pp.191-198

09/2011

DOI: 10.1111/j.1529-8027.2011.00350.x

PMCID: PMC3754828

PMID: 22003934

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Abstract

The Charcot-Marie-Tooth neuropathy score (CMTNS) is a reliable and valid composite score comprising symptoms, signs, and neurophysiological tests, which has been used in natural history studies of CMT1A and CMT1X and as an outcome measure in treatment trials of CMT1A. Following an international workshop on outcome measures in Charcot-Marie-Tooth disease (CMT), the CMTNS was modified to attempt to reduce floor and ceiling effects and to standardize patient assessment, aiming to improve its sensitivity for detecting change over time and the effect of an intervention. After agreeing on the modifications made to the CMTNS (CMTNS2), three examiners evaluated 16 patients to determine inter-rater reliability; one examiner evaluated 18 patients twice within 8 weeks to determine intra-rater reliability. Three examiners evaluated 63 patients using the CMTNS and the CMTNS2 to determine how the modifications altered scoring. For inter- and intra-rater reliability, intra-class correlation coefficients (ICCs) were ≥0.96 for the CMT symptom score and the CMT examination score. There were small but significant differences in some of the individual components of the CMTNS compared with the CMTNS2, mainly in the components that had been modified the most. A longitudinal study is in progress to determine whether the CMTNS2 is more sensitive than the CMTNS for detecting change over time.

Charcot-Marie-Tooth disease

reliability

CMT neuropathy score

Details

Title: Subtitle: Reliability of the CMT neuropathy score (second version) in Charcot-Marie-Tooth disease
Creators: Sinéad M Murphy - MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery
David N Herrmann - Department of Neurology, Division of Neuromuscular Disorders
Michael P McDermott - Department of Biostatistics and Computational Biology, University of Rochester Medical Center, Rochester, NY
Steven S Scherer - Department of Neurology, University of Pennsylvania, Philadelphia, PA
Michael E Shy - Department of Neurology, Wayne State University, Detroit, MI, USA
Mary M Reilly - MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery
Davide Pareyson - Clinic of Central and Peripheral Degenerative Neuropathies Unit, Department of Clinical Neurosciences, IRCCS Foundation, “C. Besta” Neurological Institute, Milan, Italy
Resource Type: Journal article
Publication Details: Journal of the peripheral nervous system, Vol.16(3), pp.191-198
DOI: 10.1111/j.1529-8027.2011.00350.x
PMID: 22003934
PMCID: PMC3754828
NLM abbreviation: J Peripher Nerv Syst
ISSN: 1085-9489
eISSN: 1529-8027
Grant note: U54 NS065712 || NS / National Institute of Neurological Disorders and Stroke : NINDS
Language: English
Date published: 09/2011
Academic Unit: Neurology; Molecular Physiology and Biophysics; Stead Family Department of Pediatrics; Iowa Neuroscience Institute
Record Identifier: 9984013999402771

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