Preservation of motor evoked potentials under anesthesia in children with spinal muscular atrophy type II undergoing...

Spinal muscular atrophy is a progressive condition in which movement is gradually lost as a result of the loss of spinal motor neurons. Individuals with this condition may require surgical correction of a secondary scoliosis. Motor evoked potentials were recorded using transcranial electrical stimulation in four such individuals undergoing surgery. All the patients were nonambulatory and in wheelchairs. Motor evoked potentials were recordable in both upper and lower limb muscles, with similar stimulation parameters to control subjects undergoing surgery for idiopathic scoliosis. The amplitudes of the motor evoked potentials were similar to those in control subjects, although the latencies were shorter reflective of the smaller stature of the spinal muscular atrophy patients. The relative preservation of the motor evoked potentials despite the patients' poor voluntary motor control suggests that there is a selective preservation of the motor neurons mediating the motor evoked potential in spinal muscular atrophy and a maintenance of the conduction velocities of the corticospinal tract.