Muscle velocity recovery cycles in myopathy.

OBJECTIVE: To understand the pathophysiology of myopathies by using muscle velocity recovery cycles (MVRC) and frequency ramp (RAMP) methodologies. METHODS: 42 patients with quantitative electromyography (qEMG) and biopsy or genetic verified myopathy and 42 healthy controls were examined with qEMG, MVRC and RAMP, all recorded from the anterior tibial muscle. RESULTS: There were significant differences in the motor unit potential (MUP) duration, the early and late supernormalities of the MVRC and the RAMP latencies in myopathy patients compared to controls (p < 0.05 apart from muscle relatively refractory period (MRRP)). When dividing into subgroups, the above-mentioned changes in MVRC and RAMP parameters were increased for the patients with non-inflammatory myopathy, while there were no significant changes in the group of patients with inflammatory myopathy. CONCLUSIONS: The MVRC and RAMP parameters can discriminate between healthy controls and myopathy patients, more significantly for non-inflammatory myopathy. MVRC differences with normal MRRP in myopathy differs from other conditions with membrane depolarisation. SIGNIFICANCE: MVCR and RAMP may have a potential in understanding disease pathophysiology in myopathies. The pathogenesis in non-inflammatory myopathy does not seem to be caused by a depolarisation of the resting membrane potential but rather by the change in sodium channels of the muscle membrane.

Muscle velocity recovery cycles in neurogenic muscles.

OBJECTIVE: To examine muscle membrane properties in neurogenic muscles using Muscle Velocity Recovery Cycles (MVRCs). METHODS: Forty-seven patients referred to Nerve Conduction Studies (NCS) and Electromyography (EMG) for peroneal nerve entrapment neuropathy were prospectively included. The patients were categorized as peroneal nerve entrapment neuropathy across knee (n = 22), L5-radiculapathy (n = 10), normal NCS/EMG (n = 9) and other disorders (n = 6) using NCS/EMG and neuroimaging results. Strength in anterior tibial muscle was measured by Medical Council Scale (MRC) and disease duration was recorded. In addition to conventional NCS/EMG, all subjects were examined with MVRCs in anterior tibial muscle. This provided parameters of muscle relative refractory period (MRRP) and early supernormality (ESN) and late supernormality (LSN). The results were compared with 29 age-matched healthy control subjects. RESULTS: MRRP was prolonged and ESN and LSN were reduced in neurogenic muscles. MRRP, ESN and LSN correlated to MRC and incidence of spontaneous activity but not to motor unit potential parameters or disease duration. CONCLUSIONS: MVRC changes provide in vivo evidence of depolarization in intact human muscle fibres that could underlie reduced muscle excitability and hence weakness in neurogenic muscles. SIGNIFICANCE: MVRCs appear to be a useful technique for revealing disease mechanism in a broad range of neuromuscular diseases.

MScanFit motor unit number estimation (MScan) and muscle velocity recovery cycle recordings in amyotrophic lateral sclerosis patients.

OBJECTIVE: Motor Unit Number Estimation (MUNE) methods, such as the recently developed MScanFit MUNE (MScan), may be valuable in tracking motor unit loss in ALS. Muscle Velocity Recovery Cycles (MVRCs) provide information about muscle membrane properties and can reveal disease-related changes. This study was undertaken to test the applicability of MScan to the anterior tibial muscle (TA) and to test whether the MVRCs could improve understanding of ALS pathophysiology. METHODS: Twenty-six ALS patients and 25 healthy controls were evaluated by quantitative electromyography, nerve conduction study and the two novel methods: MScan and MVRC; all in the TA and peroneal nerve. RESULTS: The estimated number of motor units for ALS patients (Median: 45, interquartile range: 28.5-76.5) was significantly lower than for the controls (117, 96.0-121.0) (P = 2.19 × 10-7). Unit size was increased only when amplitudes were expressed as percentage of CMAP. Of MVRC measurements, only relative refractory period was significantly abnormal in patients. CONCLUSION: MScanFit MUNE gives a sensitive and quantitative measure of loss of TA motor units in ALS. Muscle fiber membrane properties are mostly unaffected, despite substantial denervation, presumably due to collateral reinnervation. SIGNIFICANCE: MScan is suitable for detecting motor unit loss in TA. MVRCs do not provide new insights in ALS.

The utility of motor unit number estimation methods versus quantitative motor unit potential analysis in diagnosis of ALS.

OBJECTIVE: To compare the diagnostic utility of motor unit number estimation (MUNE) methods to motor unit potential (MUP) analysis in amyotrophic lateral sclerosis (ALS). METHODS: Twenty-five patients (1 definite, 11 probable, 9 possible ALS and 4 progressive muscular atrophy) and 22 healthy controls were prospectively included. Quantitative MUP analysis and three MUNE methods; Multiple Point Stimulation MUNE (MPS), Motor Unit Number Index (MUNIX) and MScanFit MUNE (MScan) were done in abductor pollicis brevis muscle. The sensitivities were compared by McNemar chi-square test. MUNE, MUP and revised ALS Functional Rating Scale (ALSFRS-R) parameters were correlated by regression analysis. RESULTS: The sensitivities of MPS (76%) and MScan (68%) were higher than MUP duration (36%) and amplitude (40%) in detecting motor unit loss (p < 0.05). MUNE methods increased the categorical probability from possible to probable ALS in 4 patients (16%). There was only significant correlation between ALSFRS-R and MScan (r = 0.443, p = 0.027) among the electrophysiological tests. MUNE methods did not correlate to MUP parameters. CONCLUSIONS: MUNE methods are more sensitive in showing abnormality than MUP analysis. SIGNIFICANCE: MUNE methods, in particular MScan, may have the potential to be implemented in the clinical practice for diagnosis and follow-up of neuromuscular disorders particularly ALS.