Ultima

A Smart Calculator for CMT

Charcot-Marie-Tooth disease (CMT) is the most common inherited peripheral nerve disorder, with an incidence of 1 in 2,500.

The CMTPedS Calculator is a reliable and valid clinical tool used to measure disability in children with CMT

The tool enables precise assessment of baseline performance and disease severity, monitoring of outcomes in studies over time, and responses to current or novel therapeutics.

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Components and Styles

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The CMTPedS generates a linear score of disability ranging from 0 (not affected) to 44 (severely affected) and can be completed by clinicians in about 25 minutes.

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Functional Dexterity

Functional Dexterity

Measure of hand dexterity that provides information regarding the use of the fingers and hand for daily tasks requiring 3-jaw chuck prehensions e.g. buttoning, tying shoe laces, screwing a nut and bolt.

Nine Hole Peg

Nine Hole Peg Test

Measure to examine fine motor ability, dexterity and hand/eye coordination as a measure of hand function.

Grip Strength

Grip Strength

Maximum isometric hand grip strength is measured using the Citec hand-held dynamometer.

Plantarflexion Strength

Plantarflexion Strength

Maximum isometric ankle plantarflexion strength is measured using the Citec hand-held dynamometer.

Dorsiflexion Strength

Dorsiflexion Strength

Maximum isometric ankle dorsiflexion strength is measured using the Citec hand-held dynamometer.

Pinprick

Pinprick

Neurotips are used to measure small-fiber sensation loss (A-delta fibers).

Vibration

Vibration

Rydel Seiffer tuning fork is used to measure large-fiber sensation loss (Pacinian corpuscles, A-beta fibers).

Balance

Balance

Balance subtest of the Bruininks-Oseretsky Test of Motor Proficiency, Second Edition (BOT-2) is used to measure standing and walking balance with eyes open and closed.

Gait

Gait

Measures include difficulty toe walking as an indicator of dynamic plantarflexion weakness, difficulty heel walking as an indicator of dynamic dorsiflexion weakness (and Achilles tendon shortening), and the presence of foot drop as a sign of dorsiflexion weakness during gait.

Long Jump

Long Jump

Reliable measure of power and coordination.

Six Minute Walk

Six Minute Walk

6MWT is a measure of submaximal functional endurance capacity.

For more details: See Charcot-Marie-Tooth disease Pediatric Scale equipment and training resource kit

CMTPedS Calculator

© J Burns et al

What is Charcot-Marie-Tooth disease?

Charcot-Marie-Tooth disease (CMT) is the most common inherited peripheral nerve disorder, with an incidence of 1 in 2,500. CMT is characterised by motor and sensory neuropathy progressing proximally from the extremities resulting in weakness, sensory loss, foot deformity, lateral ankle instability, problems with balance and difficulty walking. It affects children and adults and there is no curative treatment for any form of CMT, although clinical trials are increasingly occurring.

What is the CMTPedS?

The CMTPedS is a reliable and valid clinical tool used to measure disability in children with CMT. The tool enables precise assessment of baseline performance and disease severity, monitoring of outcomes in studies over time, and responses to current or novel therapeutics. The CMTPedS generates a linear score of disability ranging from 0 (not affected) to 44 (severely affected) and can be completed by clinicians in about 25 minutes.

How was the CMTPedS developed?

The Inherited Neuropathies Consortium identified a need for a patient-centred multiple-item rating scale to measure all aspects of impairment in children with CMT. The Consortium set about to build a scale, firstly generating a pool of test items that would be sufficiently sensitive and responsive to change when measuring outcomes relevant to neuropathy and disability. These items were then reviewed by expert clinicians, scientists and CMT patient representatives from across the world. Following review, a 28-item working-version scale was pilot tested with 172 participants aged 3 to 20 years with a variety of CMT types. The scale was reported as well accepted and tolerated by participants. The second stage of development required validation testing of the scale, specifically, individual item and factor analysis, reliability testing, Rasch modelling and a sensitivity analysis. The final outcome was the CMTPedS: a reliable, valid, and sensitive 11-item scale that would measure strength, dexterity, sensation, gait, balance, power and endurance in children aged 3 and above with CMT.

Publications

Key CMTPedS development and validation publication

  1. Burns J. Ouvrier R. Estilow T. Shy R. Laurá M. Pallant J. Lek M. Muntoni F. Reilly M. Pareyson D. Acsadi G. Shy M. Finkel R. Validation of the CMT Pediatric Scale as an outcome measure of disability. Annals of Neurology 71(5), 642–652, 2012.
    Publication Link: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3335189/ (Free access)

  2. Gagnon, C., Massie, R., Tremblay, M., Darcy, S., Martel, M., & Burns, J. (2017). Traduction française de l’échelle Charcot-Marie-Tooth Disease Pediatric Scale. Canadian Journal of Neurological Sciences / Journal Canadien Des Sciences Neurologiques, 2017 Aug 17:1-4. doi: 10.1017/cjn.2016.435.
    Publication Link: https://www.ncbi.nlm.nih.gov/pubmed/28814348

Related CMTPedS publications

  1. Burns J. Ouvrier R. Estilow T. Shy R. Laurá M. Eichinger K. Muntoni F. Reilly M. Pareyson D. Acsadi G. Shy M. Finkel R. Symmetry of foot alignment and ankle flexibility in paediatric Charcot-Marie-Tooth disease. Clinical Biomechanics 27(7), 744-747, 2012.
  2. Burns J. Menezes M. Finkel RS. Estilow T. Moroni I. Pagliano E. Laurá M. Muntoni F. Herrmann DN. Eichinger K. Shy R. Pareyson D. Reilly MM. Shy ME. Transitioning outcome measures: Relationship between the CMTPedS and CMTNSv2 in children, adolescents and young adults with Charcot-Marie-Tooth disease. Journal of the Peripheral Nervous System 18(2):177-80, 2013.

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Proffessor Josh Burns

Professor Josh Burns

CMT Specialist

Murdoch Childrens Research Institute

Sarva Thurai

George Charalambous

Technology Expert

Curve Tomorrow

Merryn Pearce

Merryn Pearce

CMT Project Manager

Murdoch Childrens Research Institute