Manual dexterity and related functional limitations in Hereditary Motor and Sensory Neuropathy. An explorative study

Purpose. To explore impairments in manual dexterity and perceived limitations in upper extremity-related activities in subjects with Hereditary Motor and Sensory Neuropathy (HMSN). Method. Cross-sectional study of 20 HMSN subjects. Manual dexterity was assessed using the Jebsen test of hand function. Perceived limitations were assessed using the Rehabilitation Activities Profile (RAP) and the Disabilities of Arm, Shoulder and Hand questionnaire (DASH). Results. Impaired manual dexterity was found in four out of seven Jebsen sub-tests. Turning over cards, lifting large light and large heavy objects were most impaired, as reflected by median z scores of 5.7, 12.0 and 16.9, respectively. Perceived limitations, as reflected by median and percentile (P25; P75) sum-scores, were 7.5 (3; 11.7) for the RAP domains of personal care (scale 0 - 69) and 6.0 (1.25; 15.5) for the domains of occupation (scale 0 - 42). The median (P25; P75) DASH score (scale 0 - 100) was 13.3 (2.7; 48.1). Jebsen test scores were significantly associated with RAP and DASH scores. Conclusions. Manual dexterity of HMSN subjects, especially requiring the manipulation of flat and of large objects, was impaired and associated with the amount of perceived limitations in upper extremity function. Major limitations were perceived in activities related to upper extremity function by 25% of HMSN subjects. Measurement of manual dexterity and perceived limitations should be incorporated into the evaluation and treatment of HMSN subjects.