Hand function and Disability of the Arm, Shoulder and Hand in Charcot-Marie-Tooth disease

Purpose. The aim of the present study was to examine hand function and disability in persons with Charcot-Marie-Tooth disease (CMT) and to evaluate the possible correlations between hand function and disability. Methods. Nine male, 11 female (24-73 yrs) persons with CMT in northern Sweden and a matched control group of 18 men, 22 women (21-73 yrs) participated in the study. Measurements applied were tests of dexterity (Box and Block Test; Nine-Hole Peg test), grip strength (Grippit (R)), tactile gnosis (Shape Texture Identification test) and upper-limb disability (Disabilities of the Arm Shoulder and Hand questionnaire, DASH). Results. Hand function in CMT was reduced (p<0.001) to about 60% of normal, as indicated by each of the separate outcome measures as well as by a constructed summary index of hand function. DASH score median was 38.8 (range 0-66.7) and was clearly related to hand function (r = 0.64-0.83). Conclusion. Reduced hand function in CMT was found at different dimensions according to the International Classification of Functioning, Disability and Health (ICF). We suggest that DASH can be used in persons with CMT, though clinicians should be aware that patients might score lower than expected, possibly because of a long process of adaptation when learning to live with a slowly progressive disease.