PROGRESSIVE myoclonic epilepsy was first described by Unverricht1 and Lundborg.2 It is characterized by onset in childhood or adolescence of grand mal seizures, myoclonic jerks, and dementia, all of which tend to progress ad mortem.
The disorder is often familial.
Dawson3,4 showed that evoked cortical responses to sensory stimuli were abnormal in patients with myoclonic epilepsy. The amplitude of the responses could be ten times larger than the amplitude in control individuals.
The aim of this study was to investigate the effect of some drugs on the somatonsensory evoked potentials in two patients with progressive myoclonic epilepsy.
Materials and Methods
A rectangular pulse with a frequency of 1/sec and lasting 0.5 msec was applied to the median nerve at the wrist, the strength of the stimulus being 30% higher than the motor threshold (50 to 80 v).Averaged cortical responses evoked by 20 stimuli were recorded
Gath I. Effect of Drugs on the Somatosensory Evoked Potentials in Myoclonic Epilepsy. Arch Neurol. 1969;20(4):354–357. doi:10.1001/archneur.1969.00480100030003
Artificial Intelligence Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.