Reliability of clinical outcome measures in CMT

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Neuromuscul Disord. 2007 Oct 25;

Reliability of clinical outcome measures in Charcot-Marie-Tooth

disease.

Solari A, Laurà M, Salsano E, Radice D, Pareyson D; On behalf of the

CMT-TRIAAL Study Group.

Neuroepidemiology Unit, IRCCS Foundation, " C. Besta " Neurological

Institute, Via Celoria 11, 20133 Milan, Italy.

We assessed inter- and intra-rater reliability of outcome measures

in Charcot-Marie-Tooth disease (CMT) patients. In 40 CMT patients,

we assessed reliability of Overall Neuropathy Limitations Scale

(ONLS), 10-m timed walk (T10MW), 9-hole-peg test (9-HPT), maximal

voluntary isometric contraction (MVIC) of arm (elbow flexion, hand-

grip, and three-point pinch) and leg (knee extension, foot

dorsiflexion/plantar flexion). Reliability was substantial for ONLS,

excellent for T10MW and 9-HPT. For MVIC, inter and intra-rater

reliability was excellent for hand contractions; for leg

contractions, intra-rater agreement was moderate to substantial,

whereas inter-rater agreement was poor. An ad hoc device was

produced to immobilize the foot and MVIC reliability was re-assessed

in 26 CMT patients, resulting in excellent inter-rater and intra-

rater reliability for foot dorsiflexion, and clear inter-rater

improvement for foot plantar flexion. All outcome measures appear

adequate for CMT assessment. Use of an immobilization device

improves foot MVIC reliability, preventing biased findings in

patients with greater strength.