The hypothesis of overwork weakness in Charcot-Marie-Tooth: A critical evaluation

Pomeren, Madlenka van; Selles, Ruud W.; Ginneken, Berbke T J van; Schreuders, Ton A.R.; Janssen, Wim G. M.; Stam, Henk J.

doi:10.2340/16501977-0274

Cited by 22 publications

(21 citation statements)

References 13 publications

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“…Finally, our results do not support the overwork weakness hypothesis 8,30,31,40 in subjects with CMT disease. In fact, TreSPE treatment did not determine the worsening of muscle strength or of any other outcome measure.…”

Section: Discussioncontrasting

confidence: 78%

“…These results, although partly caused by patient selection and motivation, suggest that this rehabilitative program may be applied to patients with CMT disease without fear of fatigue or overwork weakness. 30,31 Previous studies focused on the rehabilitation of NMD, including CMT, fully support our conclusion that aerobic exercise is well tolerated by these patients. 15 Indeed, we observed a slight improvement in all the outcome measures, except for the respiratory tests, after TreSPE.…”

Section: Discussionsupporting

confidence: 78%

“…This result, according to some previous data, 13Y18 suggests that aerobic exercise and rehabilitation treatment should be well tolerated by subjects with CMT disease. In fact, the patients treated with this composite program, consisting of walking at the treadmill, stretching, and proprioceptive exercises, showed a high compliance to the physical therapy, a slight (although not significant) improvement in some outcome measures, and no signs of overwork weakness 30,31 or cardiopulmonary stress.…”

Section: Discussionmentioning

confidence: 95%

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Outcome Measures and Rehabilitation Treatment in Patients Affected by Charcot-Marie-Tooth Neuropathy

Maggi

Bragadin

Padua

et al. 2011

American Journal of Physical Medicine &Amp; Rehabilitation

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Objective: We evaluated the sensitivity of various rehabilitation and lung function scales to detect differences between people with Charcot-Marie-Tooth (CMT) disease and healthy controls. We also studied whether these measurements are sensitive to disclose changes in patients with CMT disease after rehabilitative treatment.Design: Eight patients with different types of CMT participated in the study.Data were gathered at baseline; at the end of the treadmill training, stretching, respiratory, and proprioceptive exercise (TreSPE) treatment period; and after a washout period of 6 mos. The following instruments were used for data collection: Medical Research Council scale for lower limb strength; Tinetti Balance Scale; Physical Performance Battery; ankle angle, oxygen consumption, and lung function tests; peak treadmill velocity and slope; time to walk 6 m; and CMT Neuropathy Score. The participants underwent TreSPE treatment twice every week for 8 wks.Results: All rehabilitative measures were significantly worse in subjects with CMT disease than in healthy controls. Lung function was always normal except for the maximum expiratory pressure and maximum inspiratory pressure. No dropouts or worsening in any of the different outcome measures were observed after TreSPE. The ankle angle and the time to walk 6 m were the only measures that significantly improved after treatment. Conclusions:The rehabilitative outcome measures used in this protocol are useful in detecting clinical impairment in people with CMT disease. Lung function tests were confirmed to be minimally abnormal in patients with CMT disease. The TreSPE treatment, besides being safe and well tolerated, induced some training effects in CMT neuropathy, in the absence of lung function amelioration and effort tolerance. Follow-up showed that CMT patients should be treated at least twice every year because a regression of all outcome measures to the baseline state was found after a 6-mo washout period.

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Section: Discussioncontrasting

confidence: 78%

Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 95%

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Outcome Measures and Rehabilitation Treatment in Patients Affected by Charcot-Marie-Tooth Neuropathy

Maggi

Bragadin

Padua

et al. 2011

American Journal of Physical Medicine &Amp; Rehabilitation

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“…& A further issue concerns the observation that patients with chronic neuromuscular disorders may undergo overwork weakness due to overloading of the weakened muscles [91,Class IV]. Obviously, overwork may further weaken patients with CMT, but this conclusion has never been confirmed by controlled studies and has been recently denied [92,93,Class IV]. & A physical therapy program for patients with CMT should also involve muscle stretching to prevent muscle tightening and loss of strength.…”

Section: Physical Therapy and Assistive Devicesmentioning

confidence: 97%

Inherited Neuropathies

Schenone

Nobbio

Bragadin

et al. 2011

Curr Treat Options Neurol

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Inherited peripheral neuropathies are among the most common hereditary diseases of the nervous system. Charcot-Marie-Tooth (CMT) disease, also known from previous classifications as hereditary motor and sensory neuropathy (HMSN), is certainly the most common inherited neuropathy. In the past several years, various treatments for CMT have been proposed, although specific therapies are not yet available. In clinical practice, rehabilitative strategies remain the most helpful therapeutic approach to these patients. There is still a lack of consensus on the best way to rehabilitate patients affected by CMT. Based on our personal experience and on a review of the literature, we first recommend the prescription of ankle-foot orthoses (AFO) for patients affected by CMT; the choice of which patient, which AFO, and when to apply it depends on the individual condition of each patient and on the experience of the physician/therapist. Second, adaptive equipment (eg, button hook, long-handled shoehorn, elastic shoe laces) is available to compensate for hand deformities, sensory loss, and weakness. Third, moderate to intense strength training and aerobic exercise are well tolerated by patients affected by CMT; further studies are needed to establish whether these approaches are effective in improving their motor function and strength. There is not enough evidence to recommend muscle stretching exercises or proprioceptive kinesiotherapy, although in our experience both approaches may be helpful in selected CMT patients to prevent tendon retractions, muscle tightening, and loss of strength, and to improve balance. There is growing knowledge of the underlying genetic defects and molecular pathophysiology in CMT. To date, only a few clinical trials in CMT patients have been performed. A neurotrophic factor, neurotrophin 3, was used in a small sample of CMT1A patients with promising results, but it has not been tested in a larger cohort and there is currently no reason to suggest this therapy for CMT1A neuropathy. Based on positive results in an animal model of CMT1A, three trials with ascorbic acid (AA) were completed in a large number of patients with this neuropathy, with results that were negative overall. Therefore, it is not possible to recommend the use of AA in CMT1A patients at this time, but the results of a larger Italian-UK study and an American trial with higher doses of AA are still awaited. It is important to remember that a superimposed inflammatory/disimmune process may complicate the course of the neuropathy; in this case, severe worsening (especially motor) in a matter of weeks or months is a "red flag" that should suggest immunosuppressive or immunomodulatory treatment such as steroids, intravenous immunoglobulin, or plasma exchange. In fact, steroid-sensitive cases of HMSN were described many years ago, well before the genetic diagnosis was available. Symptomatic treatment to reduce neuropathic and nociceptive pain, both of which have been reported in patients affected by CMT, should be prescribed according to ...

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“…By contrast, Van Pomeren et al tested a series of 28 CMT patients (13 CMT1 and 15 CMT2) by using the conventional 6-point MRC scale and also the Rotterdam Intrinsic Hand Myometer, which allows measuring intrinsic hand muscle strength. There was no finding in favour of the OW hypothesis; rather, in CMT2 patients the key-grip test showed the DH to be stronger than the NDH 6. By using a digital handgrip dynamometer, Videler and colleagues found no significant differences between sides for grip, 2-point, tripod and lateral pinch strength in a series of 49 patients with CMT type 1A (the most common CMT type).…”

Section: Introductionmentioning

confidence: 96%

Is overwork weakness relevant in Charcot–Marie–Tooth disease?

Piscosquito¹,

Reilly²,

Schenone

et al. 2014

J Neurol Neurosurg Psychiatry

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BackgroundIn overwork weakness (OW), muscles are increasingly weakened by exercise, work or daily activities. Although it is a well-established phenomenon in several neuromuscular disorders, it is debated whether it occurs in Charcot-Marie-Tooth disease (CMT). Dominant limb muscles undergo a heavier overload than non-dominant and therefore if OW occurs we would expect them to become weaker. Four previous studies, comparing dominant and non-dominant hand strength in CMT series employing manual testing or myometry, gave contradictory results. Moreover, none of them examined the behaviour of lower limb muscles. Methods We tested the OW hypothesis in 271 CMT1A adult patients by comparing bilateral intrinsic hand and leg muscle strength with manual testing as well as manual dexterity. Results We found no significant difference between sides for the strength of first dorsal interosseous, abductor pollicis brevis, anterior tibialis and triceps surae. Dominant side muscles did not become weaker than non-dominant with increasing age and disease severity (assessed with the CMT Neuropathy Score); in fact, the dominant triceps surae was slightly stronger than the non-dominant with increasing age and disease severity. Discussion Our data does not support the OW hypothesis and the consequent harmful effect of exercise in patients with CMT1A. Physical activity should be encouraged, and rehabilitation remains the most effective treatment for CMT patients.

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The hypothesis of overwork weakness in Charcot-Marie-Tooth: A critical evaluation

Cited by 22 publications

References 13 publications

Outcome Measures and Rehabilitation Treatment in Patients Affected by Charcot-Marie-Tooth Neuropathy

Outcome Measures and Rehabilitation Treatment in Patients Affected by Charcot-Marie-Tooth Neuropathy

Inherited Neuropathies

Is overwork weakness relevant in Charcot–Marie–Tooth disease?

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