Electromyographic studies in mdx and wild-type C57 mice

Abstract

The electromyographic (EMG) characteristics of human Duchenne muscular dystrophy (DMD) have been well-described. However, to our knowledge, no prior needle electromyographic (EMG) studies of motor unit morphology have been undertaken in muscles from the mdx mouse, an animal that is genetically homologous to DMD. There are significant phenotypic differences between the human and murine dystrophic conditions, bringing into question whether the mdx mouse is an appropriate animal model for DMD. This study was done in order to characterize the EMG findings in mdx mice, compared to normal wild-type mice, and to assess for similarities to DMD. The tibialis anterior and gastrocnemius/soleus muscles from 34 mice (16 C57 wild-type and 18 mdx), divided into four age groups (3, 12, 18, and 24 months), were examined. Wild-type muscles showed normal insertional activity and no abnormal activity at rest. Motor unit action potential (MUAP) parameters were characterized. In contrast to wild-type muscles, mdx muscles showed increased insertional activity, abnormal spontaneous potentials, and the presence of complex repetitive discharges (CRDs). MUAPs showed increased numbers of phases (4.0 +/- 0.6, P < 0.001) and duration (7.1 +/- 1.2 ms, P < 0.02), as well as late components (15%). These EMG data indicate that mdx muscles display EMG characteristics similar to those found in muscles from boys with DMD, lending credence to the mdx mouse as an animal model for this disease. The data obtained in this study indicate a potential role for EMG as an in vivo, objective measurement tool that could be used longitudinally to monitor the effects of therapeutic interventions in mdx mice. This is important as there are few objective measures of muscle function in murine models that do not require killing the animal.