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

نویسندگان

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

OBJECTIVE It has been reported that the non-dominant hand of patients with Charcot-Marie-Tooth disease is stronger than the dominant hand as a result of overwork weakness. The objective of this study was to determine if this hypothesis could be verified in our population. DESIGN Survey. SUBJECTS Twenty-eight patients with Charcot-Marie-Tooth disease type I or II from a rehabilitation department of a university hospital in the Netherlands. METHODS The strength of 3 intrinsic muscle groups of the dominant and non-dominant hand were determined using the Medical Research Council scale and the Rotterdam Intrinsic Hand Myometer. Furthermore, grip strength, pinch and key grip strength were measured. RESULTS We found no differences in muscle strength for the dominant and non-dominant hand, except for a stronger key grip strength of the dominant hand in patients with Charcot-Marie-Tooth disease type II. CONCLUSION In our population, the dominant hand of patients with Charcot-Marie-Tooth disease type I and II was equally strong as the non-dominant hand, suggesting that there is no presence of overwork weakness in the dominant hand in our group of patients. This implies that patients with Charcot-Marie-Tooth disease do not have to limit the use of their hands in daily life in order to prevent muscle strength loss.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

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

BACKGROUND In 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. Fo...

متن کامل

Charcot–Marie–Tooth disease: Genetics, epidemiology and complications

Background and aims: Charcot Marie Tooth disease (CMT) is the most prevalent hereditary neuropathy and its frequency is 1 in 2500. CMT is a heterogeneous disease and has different clinical symptoms. The prevalence of CMT and involved genes differ in different countries. CMT patients experience considerable sleep problems and a higher risk of decreased quality of life. In this w...

متن کامل

Whole Exome Sequencing Revealed a Novel GJB1 Pathogenic Variant and a Rare BSCL2 Mutation in Two Iranian Large Pedigrees with Multiple Affected Cases of Charcot-Marie-Tooth

Charcot-Marie-Tooth disease (CMT) is the most common hereditary neuropathy of the peripheral nervous system with a wide range of severity and age of onset. CMT patients share similar phenotypes which make it often impossible to identify the disease types based on clinical presentation and electrophysiological studies alone. In recent years, novel genetic diagnostic approaches such as whole exom...

متن کامل

Diaphragm weakness in Charcot-Marie-Tooth disease.

Two patients are described with Charcot-Marie-Tooth disease and chronic peripheral neuropathy. Both had dyspnoea, orthopnoea, and evidence of severe diaphragm weakness. Expiratory muscle function was well preserved and abnormalities of gas exchange during sleep were only minor.

متن کامل

A Transgenic Rat Model of Charcot-Marie-Tooth Disease

Charcot-Marie-Tooth disease (CMT) is the most common inherited neuropathy in humans and has been associated with a partial duplication of chromosome 17 (CMT type 1A). We have generated a transgenic rat model of this disease and provide experimental evidence that CMT1A is caused by increased expression of the gene for peripheral myelin protein-22 (PMP22, gas-3). PMP22-transgenic rats develop gai...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Journal of rehabilitation medicine

دوره 41 1  شماره 

صفحات  -

تاریخ انتشار 2009