The Charcot-Marie-Tooth Functional Outcome Measure (CMT-FOM)

Katy Eichinger; Joshua Burns; Kayla Cornett; Chelsea Bacon; Mary Lohse Shepherd; Joan Mountain; Janet Sowden; Rosemary Shy; Michael E Shy; David N Herrmann

doi:10.1212/WNL.0000000000006323

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The Charcot-Marie-Tooth Functional Outcome Measure (CMT-FOM)

Journal article

Open access

Peer reviewed

The Charcot-Marie-Tooth Functional Outcome Measure (CMT-FOM)

Katy Eichinger, Joshua Burns, Kayla Cornett, Chelsea Bacon, Mary Lohse Shepherd, Joan Mountain, Janet Sowden, Rosemary Shy, Michael E Shy and David N Herrmann

Neurology, Vol.91(15), pp.e1381-e1384

10/09/2018

DOI: 10.1212/WNL.0000000000006323

PMCID: PMC6177280

PMID: 30232254

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Abstract

The purpose of this study was to examine the feasibility, reliability, and convergent validity of the Charcot-Marie-Tooth Functional Outcome Measure (CMT-FOM), a new performance-based measure assessing functional ability in adults with CMT disease.\nAdults with CMT type 1A (CMT1A) were recruited at the Universities of Rochester and Iowa. Participants were assessed using the CMT-FOM, CMT Exam Score (CMTES), and a symptom report. Test-retest reliability was examined using intraclass correlation coefficients, internal consistency using Cronbach α, and convergent and known-groups validity using Spearman rank analysis and the Mann-Whitney test.\nForty-three individuals (70% women; mean age 41, SD 14.9 years) participated. The CMT-FOM (mean 25.3 ± 8.7, range 12-44/52) was moderately correlated with the CMTES (ρ = 0.62;\n< 0.0001) and exhibited acceptable reliability (intraclass correlation coefficient = 0.92) and internal consistency (Cronbach α = 0.81). The CMT-FOM discriminated between participants with clinically mild vs moderate-severe CMT1A. Participants with the mildest CMT1A who demonstrated a floor effect on the CMTES showed functional limitations on the CMT-FOM.\nThe CMT-FOM is well tolerated and showed no floor/ceiling effects in an adult CMT1A cohort matching those likely to enter upcoming clinical trials. It appears to be reliable, and our data support convergent and known-groups validity in adults with CMT1A. Longitudinal studies further examining the psychometric properties of the CMT-FOM and its responsiveness to change before its application in therapeutic trials are necessary.

Feasibility Studies

Disability Evaluation

Young Adult

Reproducibility of Results

Humans

Middle Aged

Adolescent

Adult

Female

Male

Aged

Charcot-Marie-Tooth Disease - diagnosis

Details

Title: Subtitle: The Charcot-Marie-Tooth Functional Outcome Measure (CMT-FOM)
Creators: Katy Eichinger - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City. Katy_eichinger@urmc.rochester.edu
Joshua Burns - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City
Kayla Cornett - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City
Chelsea Bacon - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City
Mary Lohse Shepherd - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City
Joan Mountain - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City
Janet Sowden - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City
Rosemary Shy - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City
Michael E Shy - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City
David N Herrmann - From the Department of Neurology (K.E., J.M., J.S., D.N.H.), University of Rochester, NY; Faculty of Health Sciences & Children's Hospital at Westmead (J.B., K.C.), University of Sydney, Australia; and Departments of Neurology (C.B., M.E.S.), Physical Therapy (M.L.S.), and Pediatrics (R.S.), University of Iowa, Carver College of Medicine, Iowa City
Resource Type: Journal article
Publication Details: Neurology, Vol.91(15), pp.e1381-e1384
DOI: 10.1212/WNL.0000000000006323
PMID: 30232254
PMCID: PMC6177280
NLM abbreviation: Neurology
ISSN: 0028-3878
eISSN: 1526-632X
Publisher: United States
Grant note: U54 NS065712 / NINDS NIH HHS
Alternative title: CMT-FOM
Language: English
Date published: 10/09/2018
Academic Unit: Neurology; Molecular Physiology and Biophysics; Psychiatry; Stead Family Department of Pediatrics; Iowa Neuroscience Institute
Record Identifier: 9984070002702771

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