No abstract

We conclude that the presence of neonatal leg movements does not indicate integrity of functional lower motor neuron innervation by spinal segments caudal to the meningomyelocele. Present observations could explain why fetal surgery at the level of the meningomyelocele does not prevent loss of leg movements.

Muscle fiber conduction velocity (MFCV) estimation from surface signals is widely used to study muscle function, e.g., in neuromuscular disease and in fatigue studies. However, most analysis methods do not yield information about the velocity distribution of the various motor unit action potentials. We have developed a new method-the interpeak latency method (IPL)-to calculate both the mean MFCV and the spread of conduction velocities in vivo, from bipolar surface electromyogram (sEMG) during isometric contractions. sEMG was analyzed in the biceps brachii muscle in 15 young male volunteers. The motor unit action potential peaks are automatically detected with a computer program. Associated peaks are used to calculate a mean MFCV and the SD. The SD is taken as a measure of the MFCV spread. The main finding is that the IPL method can derive a measure of MFCV spread at different contraction levels. In conclusion, the IPL method provides accurate values for the MFCV and additionally gives information about the scatter of conduction velocities.

The relationship between muscle fibre conduction velocity (MFCV) and the power spectrum of surface EMGs in 3 human volunteers was studied during isometric contractions at 40% maximum voluntary contraction. In addition, the recovery of these two parameters was measured during short lasting contractions at the same force level every 30 s. The recovery phase was also studied during ischaemia, thereby preventing the recovery of MFCV. The mean MFCV was calculated by the cross-correlation method. The measurements were facilitated by a real-time estimation of the cross-correlation and the MFCV and by a graphic display of the digitised signal. During contraction a nearly linear relation was found between MFCV and the median frequency of the power spectrum (MPF). During recovery this relationship was lost in one subject: MPF restored much faster then MFCV. During recovery under ischemia MFCV did not recover, but MPF recovered partially in all subjects. It is concluded that the shift of the power spectrum to lower frequencies during fatigue cannot be explained by changes in MFCV alone. Central mechanisms also influence the power spectrum and studying the recovery of local muscle fatigue during ischemia may separate these influences from that of MFCV on the power spectrum during fatigue.

A sixth family with autosomal dominantly inherited myokymia and paroxysmal ataxia is described. The syndrome in this family is linked to the recently discovered locus for inherited myokymia and paroxysmal ataxia on the human chromosome 12p, and a missense mutation is shown in the KCNA1 gene.