Compound Muscle Action Potential Responses Research Articles

Congenital Myasthenic Syndrome: Presentation, Electrodiagnosis, and Muscle Biopsy

We report 10 children with congenital myasthenic syndromes diagnosed by clinical features, electrodiagnostic studies, and response to acetylcholinesterase inhibitors. Age at diagnosis (mean = 4.4 years; range 0.2–10 years) correlated with age fatigue was recognized. Symptoms at presentation included mild gross motor development delay (7/10), speech articulation difficulty (5/10), and respiratory and feeding difficulties resulting in poor growth in 7 of 10 children. None of the five children with possible presynaptic abnormalities had decremental compound muscle action potential responses to 2 Hz repetitive nerve stimulation. Instead, electrodiagnostic studies showed a more than 100% increment of compound muscle action potential amplitude during 50 Hz repetitive nerve stimulation in two children and sustained compound muscle action potential decrement to 2 Hz repetitive nerve stimulation after depletion (10 Hz stimulation for 10 min) in four children. Muscle biopsies ( n = 7) showed mild to severe variation in fiber size. Our experience suggests that many children with congenital myasthenic syndromes might be undiagnosed because of atypical presentation and because additional electrophysiologic studies are required.

Differential effects of stimulus intensity on peripheral and neuromagnetic cortical responses to median nerve stimulation

To study the differential effects of tactile stimulus intensity on cortical and peripheral responses, we measured neuromagnetic cortical responses, compound muscle action potentials (CMAP), sensory nerve action potentials (SNAP), and the subjective estimation of tactile magnitude to electric median nerve stimulation at the wrist in 13 male healthy adults. The sensory perception threshold (ST) for electric pulses at wrist skin was determined and then various levels of stimulus intensity (1 ∼ 6 ST) were given to each subject. At 1 ST, only the P50m components of the primary somatosensory (SI) cortical responses were recorded. The second somatosensory (SII) cortical responses were saturated at 2 ST, while the SI responses reached maximum at 3 ST equivalent to the subjective threshold intensity for “strong” tactility. The CMAP and SNAP were maximum at 4–5 ST. At 2 ST, >70% of maximum SI responses were produced, whereas only <40% of maximum CMAP or SNAP responses were obtained. We concluded that the stimulus intensities for activating or saturating somatosensory cortical responses were lower than those for CMAP and SNAP. The differential intensity effects on cortical and peripheral responses suggest a polysynaptic organization underlying the central amplification for somatosensory cortical activation. The optimal intensity levels for producing maximum SI and SII responses were 3 and 2 ST, respectively. Compared with the SII, the SI plays a crucial role in the coding of the tactile stimulus intensity.

Neurophysiologic Monitoring of Spinal Nerve Root Function During Instrumented Posterior Lumbar Spine Surgery

Retrospective review of 61 consecutive patients. To determine the effectiveness of combining intraoperative monitoring of both spontaneous electromyographic activity and compound muscle action potential response to stimulation for detecting a perforation of the pedicle cortex irritation of nerve root during lumbar spine fusion surgery. The complication rate from instrumentation used with lumbar spine fusion varies from 1 to 33%. To prevent neurologic complications, several monitoring techniques have been used to alert surgeons to possible neurologic damage being introduced during nerve decompression or placement of instrumentation with spine procedures. Because of different sensitivities, one monitoring technique may not be as effective for preventing complications as a combination of techniques. Sixty-one consecutive patients who underwent instrumented posterior lumbar fusions received continuous electromyographic monitoring and stimulus-evoked electromyographic monitoring. A significant neurophysiologic event was signaled by sustained neurotonic electromyographic activity, prompting an alert and a pause in the surgical manipulations that precipitated the activity. After insertion of the transpedicular screws, the integrity of the pedicle cortex was tested by stimulating each screw head and recording compound muscle action potentials. In the presence of a pedicle breach, stimulus intensities below 7 mA were sufficient to evoke compound muscle action potentials from the muscle group innervated by the adjacent spinal nerve root, prompting a surgical alert and subsequent repositioning of the screw. Fourteen significant neurophysiologic events occurred in 13 of 61 patients (21%). Sustained neurotonic electromyographic discharges occurred in 5 of 40 patients during placement of interbody fusion cages, in 2 patients during placement of transpedicular screws, and in 1 patient during tightening of rods. On pedicle screw stimulation, breaches of the pedicle cortex were detected in 6 patients. After surgery, no new neurologic deficits were found in 60 of the 61 patients. One patient who experienced temporary paraparesis had sustained neurotonic electromyographic discharges during retraction of the thecal sac and distraction of the disc space before placement of the cage. These results suggest that intraoperative electromyographic monitoring provides a real-time measure of impending spinal nerve root injury during instrumented posterior lumbar fusion, allowing for timely intervention and minimization of negative postoperative sequela.

Efficacious use of a cap shaped coil for transcranial magnetic stimulation of descending motor paths.

We report another technique of transcranial magnetic stimulation (TMS) for exciting the originating cells of the descending corticospinal tract. A cap shaped TMS coil has been described for simultaneously exciting muscles in all four extremities. This TMS coil is useful for monitoring the functional integrity of the descending motor paths during spinal cord surgery, because information regarding the integrity of both the left and right sides of the spinal cord motor paths can be obtained concurrently. Despite the improved design of the cap coil, careful placement is required for achieving bilateral spinal cord motor responses. Cortical mapping was used to identify the optimum scalp foci for the muscles studied. The cap coil must overlap these foci to simultaneously elicit compound muscle action potentials (CMAPs) in all four extremities. Increasing TMS stimulation intensity will increase the magnitude of the acquired CMAPs responses without significantly changing latency.

Electrical thresholds for biomechanical response in the ankle to direct stimulation of spinal roots L4, L5, and S1. Implications for intraoperative pedicle screw testing.

A comparison of electrical thresholds for biomechanical response in the ankle and for evoked electromyographic signals from specific leg muscles during intraoperative extradural direct stimulation of roots L4, L5, and S1. To determine whether a biomechanical response in the ankle to direct root stimulation occurs before evoked electromyographic signals and to determine differences in electrical excitability of the roots circumferentially. Stimulus intensities of 1.2-5.7 mA are reported to evoke electromyographic response in corresponding muscles to direct stimulation of normal roots. Stimulus intensities of 6-8 mA were suggested to detect bony pedicular compromise by stimulation of a hole or a screw during pedicle instrumentation. Electrical thresholds of three-dimensional torque response in the ankle to direct root stimulation have not yet been evaluated and compared with thresholds of evoked electromyogram. Direct monopolar stimulation of the surgically exposed roots L4, L5, and S1 was performed from different sites around the root by a cuff multielectrode. Biomechanical response was measured as an isometric torque in the ankle at each of three orthogonal axes. Compound muscle action potentials (CMAPs) from root-specific muscles were detected by a pair of surface or wire electrodes. Mean threshold for biomechanical response in the ankle to stimulation of roots L4, L5, and S1 was 0.72 +/- 0.39 mA and for CMAP response was 1.09 mA +/- 0.36 (N = 13). Thresholds for biomechanical responses were significantly lower than for CMAP responses (P = 0.0004; paired t test). Nerve roots were electrically most excitable on their ventral aspects. The biomechanical response in the joint to root stimulation can be used to test all root-related muscles crossing that joint at their individual innervation pattern and their residual innervation and to detect electrical excitation of the root at electric thresholds lower than those for detecting CMAP from single standard root-specific muscle. However, this method does not provide sufficient root specificity. It will be valuable in conjunction with multimodality neurophysiologic monitoring of the roots for earlier and more reliable detection of pedicle bone breakthrough or integrity. Further clinical investigations are suggested.

Effect of brachial plexus co-activation on phrenic nerve conduction time

BACKGROUNDDiaphragm function can be assessed by electromyography of the diaphragm during electrical phrenic nerve stimulation (ES). Whether phrenic nerve conduction time (PNCT) and diaphragm electrical activity can be reliably measured...

The influence of active electrode placement on CMAP amplitude

The compound muscle action potential (CMAP) is a measure of the number of axons in a nerve. Placement of the active recording electrode over the motor point of a muscle is thought to give the maximal response, but there is considerable variation in amplitude among initially negative CMAP wave forms. Ten examiners of varied training backgrounds and experience placed the active electrode as they usually do over the thenar, hypothenar, abductor hallucis, and extensor digitorum brevis muscles in the same normal subject. There was variability of the CMAP amplitude recorded over each muscle; the lowest value recorded from a muscle was 57% of the maximum value, and the lowest median value was 77%. There was no relation between examiner background or level of training and recording the maximal response. Higher amplitude CMAPs were associated with steeper wave form slopes, but the range of correlations between amplitude and slope was 0.42 to 0.92. We conclude that when it is important to record the maximal CMAP response, empirical assessment by moving the active electrode is necessary.

Safety Versus Efficacy of Spinal Cord Stimulation for the Generation of Motor-Evoked Potentials in the Rat

The relative safety and efficacy of direct versus indirect methods of spinal cord stimulation for the production of descending motor-evoked responses was studied in pentobarbital-anesthetized rats (n = 39). Electrical stimuli were delivered for 1 h, either directly to the cord dorsum using silver ball electrodes or indirectly through jeweler's screws implanted in the intact laminae. Compound muscle action potentials (CMAPs) were recorded differentially in the quadriceps and evaluated for their morphology and reproducibility. The traumatic effects of stimulation were assessed using intraoperative somatosensory-evoked potentials, blinded neurological examinations for 2 weeks postoperatively, and histopathological and neurochemical analyses in postmortem spinal tissues. In separate experiments, the neural substrates of the muscle-evoked response to indirect cord stimulation were examined. Direct, epidural stimulation of the spinal cord at intensities sufficient to elicit reproducible CMAPs consistently resulted in mild behavioral deficits (13 of 18 animals) that were accompanied by postmortem changes in spinal histology and chemistry. Some of these behavioral deficits (5 of 13 animals) were resolved at 2 weeks. There was rarely an early sign of motor or sensory conduction derangement in these animals. In 2 animals with severe behavioral dysfunction, the somatosensory-evoked response was abolished immediately after spinal stimulation. However, CMAP responses were unaltered. Examination of the strength-duration relationship for the production of threshold responses to translaminar constant current stimulation, as well as experiments using selective transection of the dorsal columns, revealed the CMAP responses to be neurally mediated and conducted through the cord independent of the ascending sensory tracts that mediate the rat's somatosensory-evoked response. Data are discussed in terms of the potential experimental usefulness of CAMPs elicited by indirect dorsal spinal stimulation.