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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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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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.
Measure to examine fine motor ability, dexterity and hand/eye coordination as a measure of hand function.
Maximum isometric hand grip strength is measured using the Citec hand-held dynamometer.
Maximum isometric ankle plantarflexion strength is measured using the Citec hand-held dynamometer.
Maximum isometric ankle dorsiflexion strength is measured using the Citec hand-held dynamometer.
Neurotips are used to measure small-fiber sensation loss (A-delta fibers).
Rydel Seiffer tuning fork is used to measure large-fiber sensation loss (Pacinian corpuscles, A-beta fibers).
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.
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.
Reliable measure of power and coordination.
6MWT is a measure of submaximal functional endurance capacity.
For more details: See Charcot-Marie-Tooth disease Pediatric Scale equipment and training resource kit
© J Burns et al
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.
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.
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.
CMT Specialist
Murdoch Childrens Research Institute
Technology Expert
Curve Tomorrow
CMT Project Manager
Murdoch Childrens Research Institute
