Involuntary painful muscle contractions in Satoyoshi syndrome: A surface electromyographic study

We report a child with Satoyoshi syndrome manifested by involuntary painful muscle contractions and alopecia. Although an autoimunne origin of Satoyoshi syndrome seems likely, its exact etiology remains as yet unknown, as is the origin of the involuntary contractions. To gain a better understanding of the electrophysiological characteristics of the involuntary contractions, we performed a surface electromyographic (EMG) study. We investigated muscle contractions in the legs using two noninvasive techniques: high-density surface EMG (HD-sEMG) recordings on one muscle, and polymyographic surface EMG (sEMG) recordings on various muscles. During the involuntary contractions, HD-sEMG showed a fourfold increase in amplitude compared to maximal voluntary contractions. These high potentials were widely distributed across the whole muscle and showed a pronounced oscillatory behavior with a frequency around 45 Rz. Polymyographic sEMG revealed that the involuntary contractions often occur simultaneously in various muscles or showed a switch of activity from one muscle to another. These findings point to hyperactivity or a disinhibition at the alpha motor neuron level, originating probably at level, although a central on in cannot be excluded. © 2006 Movement Disorder Society.