The authors prospectively studied the value of clinical and neurophysiologic measurements in assessing progression in ALS. Motor unit number estimation (MUNE) and the neurophysiologic index (NI) were significantly correlated with ADM strength (maximal voluntary isometric contraction force in the abductor digiti minimi muscle [MVIC-ADM]). MUNE and the NI were reliable, but the NI showed a lower variation. On assessing progression at 3, 6, and 12 months, MUNE, NI, and MVIC-ADM showed the highest rate of change. The NI is a potentially useful new neurophysiologic measurement.

F-wave excitability is largely dependent on the size of the lower motor neuron pool.

There is a need for a sensitive neurophysiological measure of disease progression in following the course of patients with amyotrophic lateral sclerosis (ALS). We studied two groups of nine ALS patients, one with slow progression (Group A) and the other with rapid progression (Group B). We evaluated muscle strength scores using the Medical Research Council (MRC) scale in limb and trunk muscles, forced vital capacity (FVC), and ALS functional rating scale (ALS-FRS) scores. Maximal voluntary isometric contraction (MVIC) of the abductor digiti minimi muscle (ADM) was measured, using a digital device. We also measured M-wave amplitude and area in the ADM, and the distal motor latency and F-wave frequency in the ulnar nerve; from these data, the neurophysiological index (NI) was calculated, as described previously. In both groups, the NI was the most sensitive measure of change, with the smallest coefficient of variation. We conclude that the NI, which requires no special technology and no new clinical or technical skills to use, is sensitive to change, and therefore may be useful in clinical trials, as well as in a clinical setting.

No abstract

Reliability in strength and neurophysiological measurements is important in evaluating progression of neuromuscular diseases. No data are available on the variability of neurophysiological parameters as compared with maximal voluntary isometric contraction (MVIC) in the same muscles, in healthy subjects. A control population of 26 healthy subjects was studied twice on different days. We evaluated the reliability of neurophysiological parameters obtained from bilateral ulnar nerve stimulation, recording the response over the abductor digiti minimi (ADM) muscle, including distal motor latency, compound muscle potential amplitude and area, F-wave frequency and mean F-wave latency, and the derived neurophysiological index that we have described previously. MVIC force was measured in each session in both ADM muscles. The variances between the grouped data obtained in the two recording sessions were identical, indicating a low intrinsic variability with this experimental methodology. Comparison of the mean values obtained in the two sessions revealed no statistically significant differences. The reliability of these neurophysiological and strength measurements in the same nerve/muscle system suggests they may be useful in comparing the dynamics of weakness and neurophysiological change in neuromuscular disease.