Hand strength and fatigue in patients with hereditary motor and sensory neuropathy (types I and II)

Objectives: To compare maximal isometric hand strength and fatigue between subjects with hereditary motor and sensory neuropathy (HMSN) and healthy controls and to test the reproducibility of handgrip strength (peak force of handgrip [PFgrip]) and fatigue. Design: PFgrip and the decline in PFgrip during 3 sets of 15 contractions were compared. Setting: University hospital in The Netherlands. Participants: Twenty subjects with HMSN and 20 age- and sex-matched healthy controls; 15 healthy subjects for the reproducibility part of the study. Interventions: Not applicable. Main Outcome Measures: PFgrip and the decline in PFgrip were compared by using a digital handgrip dynamometer. Two-point and lateral pinch measurements of subjects with HMSN were standardized against reference values. Reproducibility measurements were performed on 15 healthy subjects on 2 separate occasions within a 1-week interval. Results: PFgrip was significantly lower in the HMSN subjects compared with controls (P <.05). Pinch measurements also showed a large variance from average normal performance. No significant difference was found in the decline in percentage of PFgrip. Reproducibility was excellent for PFgrip (intraclass correlation coefficient [ICC]=.98; 95% confidence interval [CI],.95-99) but poor for fatigue (ICC=.62; 95% CI,.20-85). Conclusion: PFgrip and 2-point and lateral pinch in HMSN subjects were significantly reduced compared with healthy controls. Our findings indicated that the rate of decline of PFgrip during effort does not vary between group

Limited upper limb functioning has impact on restrictions in participation and autonomy of patients with hereditary motor and sensory neuropathy 1a

OBJECTIVE: To evaluate upper limb functioning, restrictions on participation and the independent contribution of upper and lower limb disability to participation in hereditary motor and sensory neuropathy 1a. DESIGN: Descriptive cross-sectional study. SUBJECTS: Forty-nine patients with hereditary motor and sensory neuropathy 1a. METHODS: Perceived upper limb functioning was evaluated using the Michigan Hand Outcomes Questionnaire and participation restrictions with the Impact on Participation and Autonomy Questionnaire. Upper and lower limb domains of Guy's Neurological Disability Scale were used to determine their impact on participation restrictions. RESULTS: Limitations in upper limb functioning were perceived by 98% of the patients. Median scores ranged between 70 points for overall hand function and 100 points for aesthetics (scale 0-100). Patients were least satisfied with dominant hand performance. Most patients (46-78%) reported their participation to be sufficient. Restrictions were reported in the domains work, family roles, and autonomy outdoors. Minor problems with restricted participation were indicated by 22-55%, severe problems by 2-12%. Upper limb functioning correlated significantly with all participation subscales. Upper limb disability was independently associated with participation restrictions, whereas lower limb disability was not. CONCLUSION: Limitations in upper limb functioning were perceived by the majority of patients with hereditary motor and sensory neuropathy 1a and strongly related to restricted participatio

Manual dexterity in hereditary motor and sensory neuropathy type 1a: severity of limitations and feasibility and reliability of two assessment instruments

OBJECTIVE: To assess the prevalence and significance of impaired manual dexterity in hereditary motor and sensory neuropathy type 1a (HMSN 1a), with the Sollerman hand function and the Functional Dexterity test, and compare the reliability and agreement of the tests. DESIGN: Descriptive cross-sectional study. SUBJECTS: Forty-nine subjects with HMSN 1a. RESULTS: Forty-six (94%) subjects had an abnormal Sollerman sum score ( <80) for the dominant hand. The most difficult subtests required finger grips such as pulp, tripod and lateral pinches. Dexterity scores of both hands were categorized as "moderately functional". Test-retest reliability was excellent for the Sollerman test, with intraclass correlation coefficients between 0.98 and 0.99 (95% confidence interval (CI) 0.97-0.99), and good for Functional Dexterity test scores with correlation coefficients between 0.83 and 0.95 (95% CI. 71-0.97). The 95% limits of agreement between Sollerman tests showed that differences greater than 3 points can be interpreted as a change in dexterity. The Functional Dexterity test limits were wide. CONCLUSION: Impaired manual dexterity is common among subjects with HMSN 1a, stressing that the evaluation of dexterity is an essential element of the functional assessment. Both tests are able to detect impaired manual performance in HMSN 1a. For monitoring of disease progression and the effects of treatment programmes the Sollerman test is most suitabl

Outcomes of an exercise program in patients with dorsal or volar midcarpal laxity:a cohort study of 213 patients

PURPOSE: Describing the outcomes of an exercise program on wrist and hand function for patients with midcarpal instability (MCI).MATERIALS AND METHODS: This study has a prospective cohort design. Two hundred and thirteen patients with MCI were included. The intervention was a 3-month exercise program consisting of hand therapy and home exercises. The primary outcome was perceived wrist and hand function evaluated with the Patient-Rated Wrist/Hand Evaluation (PRWHE) three months after treatment onset. Secondary outcomes were conversion to surgery, pain, and satisfaction with treatment results.RESULTS: PRWHE total scores improved from 51 ± 19 (mean ± SD) to 33 ± 24 at 3 months (95% CI: 36-30, p &lt; 0.001). All visual analog scales for pain demonstrated clinically relevant improvements at 6 weeks and 3 months ( p &lt; 0.001). At 3 months, 81% of the participants would undergo the treatment again. After a median follow-up of 2.8 years, 46 patients (22%) converted to surgery. CONCLUSIONS: We found clinically relevant improvements in hand and wrist function and pain. Most participants would undergo treatment again and 78% of the participants did not convert to surgery. Hence, non-invasive treatment should be the primary treatment choice for patients with MCI.</p