Decay of postexercise augmentation in the Lambert-Eaton myasthenic syndrome: effect of cooling

The effect of local cooling on surface recorded compound muscle action potential (CMAP) amplitude was studied in five patients with the Lambert-Eaton myasthenic syndrome (LEMS). The time course of decay of postexercise augmentation of CMAP amplitude characteristically seen in patients with LEMS was determined. We recorded the CMAP from abductor digiti minimi (ADM) in response to supramaximal stimulation of the ulnar nerve. Thirty consecutive stimuli were delivered at 1 Hz immediately after a 10-second period of maximal voluntary contraction. Skin surface temperature was recorded throughout. Initial testing at approximately 30 degrees C was repeated after cooling the hand and forearm by 6 to 12 degrees C. The effects of blood flow on temperature were counteracted by the application of a sphygmomanometer cuff, inflated above systolic blood pressure. The CMAP amplitude following contraction decayed in an exponential manner both during warm and cold conditions. The mean time constant for decay (1/b) in all patients was increased by approximately 25% after cooling. This prolongation of the period of postexercise augmentation of CMAP amplitude in LEMS after cooling concurs with patient reports of symptomatic improvement in cold weather. The mechanism for this benefit is thought to be due to reduction in the rate of removal of calcium ions from the nerve terminal following stimulation, similar to that seen in animal models of short-term synaptic enhancement.