M C Kiernan1, I K Hart, H Bostock. 1. Sobell Department of Neurophysiology, Institute of Neurology, Queen Square, London WC1N 3 BG, UK.
Abstract
OBJECTIVES: Measures of nerve excitability provide information about biophysical properties of peripheral axons in disease states. One measure, the strength duration time constant (tau(SD)), was previously reported to be prolonged in motor axons of patients with acquired neuromyotonia. The present study used a new protocol that applies a more comprehensive and sensitive panel of measures of axonal excitability, to determine firstly whether changes in tau(SD) were present in a group of patients with evidence of spontaneous motor unit activity; and secondly, if such changes in tau(SD) were present, whether other parameters of axonal excitability were affected, to clarify the mechanism of the change in tau(SD). METHODS: Eleven patients with both symptoms and EMG evidence of spontaneous motor unit activity were studied. Eight patients had autoimmune associated acquired neuromyotonia (aNMT) and three had the cramp fasciculation syndrome. The protocol first measured stimulus-response behaviour using two stimulus durations (from which the distribution of strength-duration time constants was estimated), and then threshold tracking was used to determine threshold electrotonus to 100 ms polarising currents, a current-threshold relation (indicating inward and outward rectification), and the recovery of excitability after supramaximal activation. RESULTS: The results were compared with previously published normal data. The value for tau(SD) of motor axons in the patient group was 0.43 (0. 02) ms (mean (SEM)), identical with the control value. Most other indices of axonal excitability, including those dependent on fast potassium channels, were also found to be normal. When compared with age matched controls however, the patients with acquired neuromyotonia had significantly greater late subexcitability after an impulse, greater excitability overshoots after depolarisation or hyperpolarisation, and more accommodation. CONCLUSIONS: No clear evidence for the mechanism of ectopic discharge in these patients was obtained, probably because the activity was generated focally, and most often at the motor nerve terminals. The unexpected finding of increased excitability overshoots and accommodation compared with age matched controls, suggests a relative up regulation of slow potassium conductance, possibly as a consequence of the continuous motor unit activity.
OBJECTIVES: Measures of nerve excitability provide information about biophysical properties of peripheral axons in disease states. One measure, the strength duration time constant (tau(SD)), was previously reported to be prolonged in motor axons of patients with acquired neuromyotonia. The present study used a new protocol that applies a more comprehensive and sensitive panel of measures of axonal excitability, to determine firstly whether changes in tau(SD) were present in a group of patients with evidence of spontaneous motor unit activity; and secondly, if such changes in tau(SD) were present, whether other parameters of axonal excitability were affected, to clarify the mechanism of the change in tau(SD). METHODS: Eleven patients with both symptoms and EMG evidence of spontaneous motor unit activity were studied. Eight patients had autoimmune associated acquired neuromyotonia (aNMT) and three had the cramp fasciculation syndrome. The protocol first measured stimulus-response behaviour using two stimulus durations (from which the distribution of strength-duration time constants was estimated), and then threshold tracking was used to determine threshold electrotonus to 100 ms polarising currents, a current-threshold relation (indicating inward and outward rectification), and the recovery of excitability after supramaximal activation. RESULTS: The results were compared with previously published normal data. The value for tau(SD) of motor axons in the patient group was 0.43 (0. 02) ms (mean (SEM)), identical with the control value. Most other indices of axonal excitability, including those dependent on fast potassium channels, were also found to be normal. When compared with age matched controls however, the patients with acquired neuromyotonia had significantly greater late subexcitability after an impulse, greater excitability overshoots after depolarisation or hyperpolarisation, and more accommodation. CONCLUSIONS: No clear evidence for the mechanism of ectopic discharge in these patients was obtained, probably because the activity was generated focally, and most often at the motor nerve terminals. The unexpected finding of increased excitability overshoots and accommodation compared with age matched controls, suggests a relative up regulation of slow potassium conductance, possibly as a consequence of the continuous motor unit activity.
Authors: J R Schwarz; G Glassmeier; E C Cooper; T-C Kao; H Nodera; D Tabuena; R Kaji; H Bostock Journal: J Physiol Date: 2006-03-09 Impact factor: 5.182
Authors: Susan E Tomlinson; S Veronica Tan; Dimitri M Kullmann; Robert C Griggs; David Burke; Michael G Hanna; Hugh Bostock Journal: Brain Date: 2010-11-23 Impact factor: 13.501