Motor unit recruitment in myopathy: The myopathic EMG reconsidered

Abstract

Motor unit recruitment is abnormal in myopathies. We have addressed this subject by recording motor unit potentials (MUPs) using a standard concentric needle electrode in tibialis anterior muscles of clinically normal strength in a group of patients with myopathy (15 with myositis and 4 with facioscapulohumeral muscular dystrophy Type 1). In each recording site, a minimal voluntary contraction was sought in order to activate only 2 MUPs. At least 5 pairs of MUPs were recorded in each muscle. We analysed the recruitment rate of the first activated MUP and the mean consecutive difference (MCD) of firing frequency between the individual MUPs of each recruited pair. Results were compared with 30 healthy control subjects. In myopathy the first recorded MUs fired at similar rates to controls (8.2 vs 8.0 Hz, respectively), but the MCD of the firing rate difference between the first two recruited MUPs was less than in controls (median difference 1.78 Hz vs median difference 2.47 Hz, p = 0.02). This change suggests increased lower motor neuron excitability as a functional adaptation, since muscle strength was normal in the studied muscles. These findings are consistent with spinal cord adaptation to the functional changes associated with myopathic muscle disease, although a primary muscle fibre feedback sensing mechanism could also be involved.