The occurrence of the overwork weakness (OW) in Charcot-Marie-tooth (CMT) disease has been debated for a long time. Especially at the hands level, it is still unclear as to whether OW occurs. Contrasting results may relate to the different muscle groups evaluated and the instruments used. We concentrated to the upper limbs (UL). We recruited 120 subjects, 60 CMT patients and 60 normal controls and evaluated the strength of the tripod pinch and of the hand-grip with a dynamometer, the opposition ability with the thumb opposition test (TOT) and applied an innovative instrumental testing of hand function using the sensor engineered glove test (SEGT), which previously demonstrated its sensitiveness to measure severity of hands dysfunction in CMT patients. In CMT patients, TOT scores were significantly higher in the non-dominant hand (NDH) compared to dominant hand (DH), strength in the NDH was slightly but not significantly better than the DH. Finally, SEGT results were similar between the NDH and DH, whereas in normal controls the DH performed better. In conclusion, this study supports the existence of the overwork weakness in CMT. We can speculate that the dexterity and overall ability of the hands appear more impaired in the DH as a result of a weakness and incapacity of opposition. Our results support the importance of avoiding supramaximal exercises and educating patients to prevent incorrect movements.
ObjectiveCharcot-Marie-Tooth neuropathy affects mainly and early the lower limbs, but hands deformities are a relevant problem, which involves the quality of life of the patients. Unfortunately, there are few studies about the evaluation of the upper limbs and very rare works about the rehabilitation. A treatment study at the moment is missing and it is important to search rehabilitation exercises to improve the dexterity and the quality of life of the patients.MethodsWe recruited 9 patients with clinical and genetic diagnosis of CMT and we proposed a rehabilitation protocol which includes muscle recruitment, stretching and proprioceptive exercises for the hand with the duration of 4 weeks (two sessions for week). We evaluated the patients before and one week after the treatment with Thumb Opposition Test, Sollerman Hand Function Scale, dynamometry (tripod pinch and hand grip).ResultsThe rehabilitation protocol has been well tolerated and there were not dropouts. We did not observe any worsening in every scale we used. Every parameter tested showed an improvement especially in the right/dominant hand.ConclusionThis study demonstrates that this three phases treatment is well tolerated by patients, it is not detrimental for the hands status and perfectly reproducible by professionals. Moreover, this could be the basis for future randomized single blind projects.
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