Short- and long-latency tibial somatosensory evoked potentials in cerebral lesions affecting rolandic leg areas

Summary Short- and long-latency tibial somatosensory evoked potentials (SEPs) were studied in nine patients with clinical presentation primarily involving one lower extremity. In group 1, with extensive infarcts in the territory of anterior cerebral artery, tibial cortical SEPs were uniformly absent. In group 2, with small infarcts involving Rolandic leg areas, tibial SEPs showed a decrease in overall response amplitude and attenuation of P40. In group 3, with discrete mass lesions compressing Rolandic leg areas, P40 was preserved but might be delayed. Late SEP components (N75, P100 and N135) tended to be preserved in the patients of group 2 and 3. The data suggest that Rolandic leg areas and the neighboring cortex are crucial for short- and long-latency tibial cortical SEPs and that small lesions affecting Rolandic leg areas tend to affect short-and mid-latency SEP components.     

Effect of electroconvulsive shock on the somatosensory evoked potential in the rat

The effect of electroconvulsive shock (ECS) on the cortical somatosensory evoked potential (SEP) was studied in 25 male rats. SEPs were recorded after stimulation of the contralateral forepaw. Animals were curarized and artificially ventilated but not anesthetized. The magnitude of the ECS was 80 mA for 600 ms which produced tonic-clonic convulsions lasting an average 54 s in noncurarized control animals. SEPs were recorded during the ictal period and then at intervals for 20 min. ECS initially caused the total abolition of all components of the SEP implying a significant but transient effect on activity propagated in specific and also possibly diffuse somatosensory pathways. The reappearance of the SEP coincided with the cessation of convulsive movements and the return of the corneal reflex. The return of the waveform to near baseline condition corresponded to the regaining of the righting reflex at approximately 3 min in the control animals. The most persisting change in the SEP waveform was in the shape of the late high-amplitude component (N2) which may reflect activity in the reticular formation. Following ECS, N2 reappeared with a peak latency notably decreased (approximately 1 ms) in comparison with the mean baseline recording. There was also an attenuation in the amplitude of N2 which remained significantly depressed (at less than 50% of mean baseline amplitude) throughout the post-ECS recording period. The results are compared with the relatively few studies of the acute effects of ECS on evoked potentials in psychiatric patients and also with a neuroendocrine theory which argues that the principal site and mode of action of ECS lies in the diencephalon.     

Negative myoclonus in Creutzfeldt-Jakob disease.

OBJECTIVE: To describe electrophysiological findings in a patient with Creutzfeldt-Jakob disease (CJD) showing negative myoclonus. METHODS AND RESULTS: We studied this CJD patient electrophysiologically, in comparison with two patients with cortical reflex positive myoclonus due to benign adult familial myoclonic epilepsy (BAFME). Spontaneous negative myoclonus was associated with periodic synchronous discharges (PSDs) on the electroencephalogram, but negative myoclonus could also be induced by electrical stimulation of the median nerve in the CJD patient. This patient showed giant somatosensory evoked potentials (SEPs) and enhanced C reflexes, and the duration of the induced EMG silences was found to be significantly correlated with the amplitude of cortical SEPs. The duration of silent periods (SPs) produced by magnetic stimulation of the motor cortex was extremely long. The study of recovery function of SEPs suggested that the excitability of the somatosensory cortex was decreased during a long post-stimulus period. These findings were clearly different from those of patients with BAFME. CONCLUSIONS: This CJD patient had two types of negative myoclonus; one was associated with PSDs and the other was cortical reflex negative myoclonus. The long-lasting decrease in excitability of the sensorimotor cortices after stimulation could be related to the occurrence of both types of negative myoclonus.     

Median and tibial somatosensory evoked potentials. Changes in short- and long-latency components in patients with lesions of the thalamus and thalamo-cortical radiations

Alterations in short- and long-latency components of median and tibial somatosensory evoked potentials (SEPs) were studied in patients with lesions in the thalamus and thalamo-cortical radiations. When the lesions were located primarily in the ventro-posterior thalamus, the SEP changes consisted of the following combination: absence of response; decrease in response amplitude; delay in peak latency; and attenuation of median N20-P25 and tibial P40. The laterally situated ventro-posterior lesions tended to preferentially affect tibial SEPs whereas the medially situated lesions tended to preferentially affect median SEPs. The lateral thalamic lesions affected primarily the long-latency SEP components, whereas the medial thalamic lesions affected primarily the mid-latency or the mid- and long-latency SEP components. Corona radiata infarcts produced SEP changes similar to those with the ventro-posterior thalamic lesions except that absence of evoked responses was not observed. Subcortical infarcts tended to affect the mid- and long-latency SEP components with relative preservation of the short-latency components. The present data indicate that only the lesions involving the primary thalamic relay area affected all SEP components, particularly the short-latency components, and that the lesions in other thalamic areas can also influence the SEPs, particularly the mid- and long-latency components. The present study further demonstrates that a combined use of median and tibial SEPs is useful in delineating the topographic organization of the somatosensory system in the thalamus.     

Motor and somatosensory evoked potentials in Autosomal Dominant Hereditary Spastic Paraparesis (ADHSP) linked to chromosome 2p, SPG4

The aim of our study was to evaluate Motor Evoked Potentials (MEPs) and cortical excitability, using Transcranial Magnetic Stimulation (TMS) as well as short latency Somatosensory Evoked Potentials (SEPs) in Autosomal Dominant Hereditary Spastic Paraparesis (ADHSP) patients. MEPs were recorded from upper and lower limb muscles in 12 patients (7 m and 5f) affected by ADHSP with spastin mutation (SPG4). We measured: (i) motor threshold (MTh); (ii) total motor conduction time (TMCT); (iii) direct and indirect central motor conduction time (d-CMCT and i-CMCT) calculated by subtracting from the cortical latency those obtained on magnetic spinal stimulation (d-PMCT) and via the F-wave method (i-PMCT); (iv) MEP amplitude (MEP/Mmax ratio%) and (v) duration of the cortical silent period (CSP). Latency, amplitude and persistence of the F-wave obtained with electrical nerve stimulation were also considered; H reflex was also tested from lower extremities. SEPs were recorded from spine and scalp sites following median and posterior tibial nerve stimulation; conventional latency and amplitude measurements were performed. In a comparison with the control group, the MTh recording from lower limbs was significantly higher (67.5 +/- 7.7% versus 52.5 +/- 6.9%), MEPs were absent in one case and showed reduced amplitude in the remainders (22.9 +/- 12.6% versus 66.3 +/- 25.9% of M wave); TMCT resulted to be abnormal (36.5 +/- 3.9 ms versus 27.1 +/- 1.4 ms) and d-CMCT as well as i-CMCT were significantly prolonged (23.1 +/- 3.5 ms versus 13.8 +/- 1.3 ms; and 20.1 +/- 3.4 ms versus 10.6 +/- 1.3 ms, respectively). The CSP, which was normal from the hands, was significantly shortened from the legs and correlated with spasticity scoring (Ashworth scale). Cortical SEPs from lower limbs were abnormal in all cases, whereas SEPs by stimulation of median nerves were normal; F-wave parameters from upper limbs showed no abnormalities, whereas an increased persistence was detected from lower limbs; H reflex amplitudes resulted larger compared with controls. Moreover, shortening of the CSP, being correlated with the Ashworth scale, can be considered an electrophysiological marker of spasticity that seems to arise from impairment of the supraspinal or intracortical inhibitory pathways with an additional contribution of increased segmental motor neuron excitability. These data prove the existence of comparable neurophysiological abnormalities in ADHSP with spastin mutation (SPG4) when long ascending and descending pathways are involved.     

On the significance of giant somatosensory evoked potentials in cortical myoclonus.

Four patients with cortical myoclonus were studied. All had reflex muscle jerking and grossly enlarged somatosensory evoked responses (SEPs) following electrical stimulation of the digital nerves. In addition, three of the patients had spontaneous or action-induced myoclonus. Back-averaging the EEG from these spontaneous muscle jerks showed a large positive wave over the contralateral somatomotor cortex which preceded the jerk by about 20 ms. Administration of lisuride (0.1 mg iv) reduced the severity of the reflex and spontaneous myoclonus, but had no effect on, or increased the size of the SEP. Two of the patients also received 1 mg clonazepam iv. As with lisuride, the severity of myoclonic jerking was reduced although the size of the SEP was increased. It is concluded that the usual association between giant SEPs and reflex muscle jerking can be abolished by acute administration of lisuride and clonazepam in patients with cortical myoclonus.     

Primary progressive limb-kinetic apraxia: a case report with an electrophysiological study]

We report a 53-year-old right handed woman with a 5-year history of slowly progressive clumsiness of her right hand. Neurologic symptoms was otherwise unremarkable except for mild dysarthria. Brain CT and MRI revealed a focal atrophic change in the left precentral gyrus. She was thought to have the primary progressive limb-kinetic apraxia. Electrophysiological studies were performed to explore physiologic mechanism of her apraxia. Surface EMG revealed co-contraction of antagonistic muscles in her right upper extremity with rhythmic myoclonic discharges. C-reflex was positive after median nerve stimulation only on the affected side. SEPs elicited by the median nerve stimulation were not enlarged and the SEP recovery curves showed no abnormal facilitation or inhibition. In addition, the premyoclonus spike was demonstrated by Jerk-locked averaging. Transcranial magnetic stimulation using double pulse paradigm revealed a decrease in the level of cortico-cortical inhibition on the motor cortex in the affected side. Median nerve stimulation given prior to the transcortical magnetic stimulation on the size of the magnetic evoked potential (MEP) revealed abnormal facilitations on the affected side, especially at conditioning-test interval of 60-80 ms. Therefore, our results indicate increase in the excitability of motor cortical neurons in primary progressive limb-kinetic apraxia, likely due to a decreased excitability of cortico-cortical inhibitory mechanism as a result of focal degeneration of cortical neurons.     

Age-related changes of cortical excitability in subjects with sleep-enhanced centrotemporal spikes: a somatosensory evoked potential study.

Middle-latency somatosensory evoked potentials (SEPs) of particularly large amplitude (giant) have been reported in subjects with benign childhood epilepsy with centrotemporal spikes (BECT) and in normal children, which usually show significant age-related changes. However, the mechanisms by which age modifies the appearance of centrotemporal spikes and giant SEPs in these children, are not known. The characteristics of SEPs were studied in a group of 18 subjects (10 males and 8 females, aged 7.1-17.2 years) with sleep-enhanced centrotemporal spikes, with or without BECT and the results were compared with those obtained from a group of age-matched normal controls. Giant SEPs were recorded in 6 subjects and, in 3 of these, EEG spikes evoked by hand tapping were obtained also. No subjects with giant SEPs were found in subjects older than 12 years, and an age-related decrease in amplitude of giant SEPs as this age approached was observed. Moreover, at repeated SEP recordings, a clear trend towards a more important reduction in amplitude of giant SEPs over the temporal and parietal than over the central regions was evident. The study of EEG spikes evoked by hand tapping showed a striking similarity between the averaged evoked spikes and the main negative component of giant SEPs. It was also possible to observe that the spike negative peak recorded over the central areas always preceded the same component recorded over the parietal and temporal areas by 5-15 ms. Our study seems to support the idea that giant SEPs in subjects with centrotemporal spikes are generated by a complex mechanism different from that at the basis of the normal N60 component of SEPs; they also show peculiar age-related modifications which can be interpreted in terms of maturational changes of brain excitability/inhibition and probably constitute a tool for monitoring the clinical course of BECT, when present.     

A case of clinically diagnosed corticobasal degeneration with unilateral cortical reflex myoclonus showing so-called giant SEP]

We report a 66-year-old woman clinically diagnosed as having a corticobasal degeneration (CBD), who showed electrophysiologically cortical reflex myoclonus. She developed a clumsiness and action myoclonus on the right extremities, and aphasia. The extrapyramidal signs such as dystonia and rigidity were also noted on the right side. Sequential MR images showed a progressive brain atrophy in the left frontoparietal area, where a blood perfusion was reduced on single photon emission computed tomography (SPECT). The median nerve stimulation on the affected right side, but not left side, elicited an enhanced long-loop reflex. The onset latency of the long-loop reflex (43.8msec) was similar to that of the reported cases of CBD (Thompson et al, 1994); but, significantly shorter than that reported in the patients with typical cortical reflex myoclonus. The right median nerve stimulation also elicited so-called giant somatosensory evoked potentials (SEPs). On the basis of the scalp topography of the giant SEPs, we found the high amplitude central P22-N30 components to reflect a radial dipole. We also recorded the myoclonus-related cortical spike by jerk-locked back averaging. Both the giant SEP and myoclonus-related cortical spike were recorded only on the left scalp. We therefore suggest that these two cortical activities are similar in terms of wave form, scalp topography and time relationship to either the long-loop reflex or myoclonus and may be located in the precentral area. This is the first report of a patient with CBD presenting both the giant SEP and myoclonus-related cortical spike.     

Evidence of phase-dependent nociceptive reflexes during locomotion in man

In 10 healthy subjects freely walking along a straightline, the effects of painful sural nerve stimulation, applied in different phases of the step cycle, were investigated on two antagonistic muscles of the ipsilateral lower limb acting on the knee joint: vastus lateralis (VL) and biceps femoris caput breve (BF). A clear-cut modulation in the amplitude (area) of the net reflex responses was consistently observed in both the motor nuclei explored. The extensor muscle, VL, exhibited a long-latency (mean 122 ms) reflex response, which was maximally increased by stimuli applied toward the end of the swing and in the first half of the stance phase of the stride, whereas the response appeared to be gated during the transition from the foot-flat to forefoot-contact phase. A second facilitation period was brought about by stimuli delivered in the early swing. When the response occurred superimposed on the VL locomotor activity, suppression of the ongoing EMG preceded the reflex discharge. In the flexor, BF, the same stimulus elicited a short-latency (mean 57 ms) and a long-latency (mean 132 ms) reflex response. The former was maximal after stimulation around the toe-off phase and the latter was strikingly enhanced in the late swing, where it was preceded by suppression of the background locomotor EMG activity. Responses with intermediate features (latency 70 to 80 ms, duration 90 to 120 ms), probably resulting from the merging of the early and late components, might be evoked in addition, being greatest in the last swing and in the period preceding toe-off. The findings show that in man the reflex pattern evoked by a painful cutaneous stimulus during locomotion is determined by the phase of the step cycle during which the stimulus is delivered. A functional role in maintenance of postural balance during destabilizing withdrawal reactions is conceivable.     

Somatosensory evoked potentials following stimulation of the lower limb in cortical reflex myoclonus.

Generating mechanisms of giant somatosensory evoked potentials (SEPs) following stimulation of the posterior tibial nerve as well as the big toe were investigated in three patients with cortical reflex myoclonus. Scalp distributions of recognisable components were very similar to those in normal subjects, except that their amplitude was much larger. The tibial nerve SEPs were remarkably attenuated by interfering tactile stimulation. Therefore, the giant SEPs observed in the present cases seem to be, at least partially, due to input from cutaneous nerve fibres on the background of extremely enhanced excitability in area 3b of the primary sensory cortex where normal SEPs are generated.     

Generators and temporal succession of giant somatosensory evoked potentials in cortical reflex myoclonus: epicortical recording from sensorimotor cortex.

OBJECTIVE: To clarify the generator mechanism of giant somatosensory evoked potentials (giant SEPs) and the hyperexcitability of primary somatosensory and motor cortices (SI and MI). METHODS: In a patient with intractable focal seizures manifesting cortical reflex myoclonus of the left foot, giant SEPs to left tibial nerve stimulation were epicortically recorded as a part of presurgical evaluation with subdural electrodes. RESULTS: In the single pulse SEPs, enlarged P1-N1 components were observed at the foot area of the SI and MI (86.5-258.8 microV, respectively), and the peak latencies were always shorter at SI than at MI by 6 ms. Similar findings were obtained for peroneal and sural nerve stimulation. In the paired pulse SEPs, the second response was less suppressed, as compared to other interstimulus intervals (ISIs), with ISIs of 40 and 200 ms both at SI and MI. CONCLUSIONS: In this particular patient, cortical hyperexcitability to somatosensory stimuli seems to originate from SI but subsequently both SI and MI are responsible for the generation of giant SEPs and cortical reflex myoclonus. SIGNIFICANCE: Somatosensory and primary motor cortices both generated enhanced early cortical components of SEPs, most likely by enhancing the latter by the former.     

Cortical tremor: a variant of cortical reflex myoclonus

Two patients with action tremor that was thought to originate in the cerebral cortex showed fine shivering-like finger twitching provoked mainly by action and posture. Surface EMG showed relatively rhythmic discharge at a rate of about 9 Hz, which resembled essential tremor. However, electrophysiologic studies revealed giant somatosensory evoked potentials (SEPs) with enhanced long-loop reflex and premovement cortical spike by the jerk-locked averaging method. Treatment with beta-blocker showed no effect, but anticonvulsants such as clonazepam, valproate, and primidone were effective to suppress the tremor and the amplitude of SEPs. We call this involuntary movement "cortical tremor," which is in fact a variant of cortical reflex myoclonus.     

Cortical reflex myoclonus associated with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS): a case report.

A 9-year-old female MELAS patient with myoclonus is reported, with emphasis on the results of electrophysiological studies of the myoclonus. At age 5 years she experienced a stroke-like episode, and a diagnosis of MELAS was made at age 6 years on the basis of muscle biopsy findings. At age 9 years spontaneous and segmental myoclonus, predominantly affecting the upper extremities, developed because of complications. Electrophysiological examination, including of somatosensory-evoked potentials (SEPs) and averaged EMG for long loop reflexes, revealed so-called "giant SEP" and enhanced long loop reflexes reflecting cortical hyperexicitability. Jerk-locked averaging yielded no myoclonus related spikes, but myoclonus-contingent 4-5 Hz theta bursts appeared. These findings suggest that some types of MELAS may be associated with cortical types of myoclonus.     

Central nervous system abnormalities in vaginismus

ObjectiveTo investigate possible altered CNS excitability in vaginismus.MethodsIn 10 patients with primary idiopathic lifelong vaginismus, 10 with vulvar vestibulitis syndrome accompanied by vaginismus and healthy controls we recorded EMG activity from the levator ani (LA) and external anal sphincter (EAS) muscles and tested bulbocavernosus reflex (BCR). Pudendal-nerve somatosensory evoked potentials (SEPs) were tested after a single stimulus. Pudendal-nerve SEP recovery functions were assessed using a paired conditioning-test paradigm at interstimulus intervals (ISIs) of 5, 20 and 40 ms.ResultsEMG in patients showed muscular hyperactivity at rest and reduced inhibition during straining. The BCR polysynaptic R2 had larger amplitude (p < 0.01) and longer duration (p < 0.01) in patients from both groups than in controls. In controls, paired-pulse SEPs were suppressed at the 5 ms ISI for N35–P40 (p < 0.05) and P40–N50 ms (p < 0.001) and facilitated at the 20 ms ISI for N35–P40 (p < 0.05) and P40–N50 (p < 0.05). No significant differences were found in the paired-pulse N35–P40 in patients and controls but the cortical P40–N50 at 20 ISI was facilitated in patients (p < 0.05).ConclusionsEMG activity is enhanced and the cortical SEP recovery cycle and BCR are hyperexcitable in vaginismus.SignificanceThe neurophysiological abnormalities in patients with vaginismus indicate concomitant CNS changes in this disorder.     

Operant conditioning of somatosensory evoked potential (SEP) amplitude in rats. I. Specific changes in SEP amplitude and a naloxone-reversible somatotopically specific change in facial nociception

The aim of this experiment was to investigate possible endogenous opioid modulation of innocuous somatosensory activity. Somatosensory activity was measured by recording cortical somatosensory evoked potential (SEP) and reflex movement amplitude evoked by innocuous electrical stimulation of the spinal trigeminal tract in awake rats. Putative endogenous opioid activity was blocked using the opiate antagonist naloxone (1 mg/kg). The amplitude of midlatency SEP components (14-50 ms latency) increased following administration of naloxone and repeated stimulus presentations. The amplitude of these components decreased following administration of the opiate agonist morphine (3 mg/kg). An early cortical component (10 ms latency) habituated following the administration of saline but did not habituate following naloxone. Naloxone also enhanced habituation of the late SEP components (60-120 ms latency) and reflex movement evoked at higher stimulus intensities. Morphine decreased the amplitude of the early cortical component but had no consistent effect on the amplitude of the late SEP components.     

Electrodiagnostic findings in syringomyelia

Electrodiagnostic abnormalities are well known to occur in syringomyelia although the findings are nonspecific. The objective of this work was to describe different types of spontaneous electromyographic (EMG) activity and reflex responses, which may be useful and more specific than conventional findings for the electrodiagnosis of syringomyelia. We studied 43 patients with syringomyelia by four-channel surface EMG and by recording the long-latency responses to distal stimulation of the median and tibial nerves. Continuous motor unit activity (CMUA) was found in 18 patients, synchronous motor unit potentials (SMUP) in 10, respiratory synkinesis (RS) in 5, and myokymic discharges in 4. Long-latency responses (LLR) with latencies ranging from 55 to 150 ms were found in 14 patients. Patients with syringomyelia thus show a wide variation of spontaneous EMG activity. An increase in excitability of spinal motor neurons is probably the basic underlying mechanism.     

Somatosensory evoked potentials in the evaluation of lumbosacral radiculopathy.

We performed lower extremity somatosensory evoked potential (SEP) studies in 59 patients with signs or symptoms suggestive of lumbosacral radiculopathy and compared them with results of myelography with post-myelogram CT (myelogram/CT), MRI, and other electrodiagnostic studies. Of 38 patients with abnormal myelogram/CTs, 32 had abnormal SEPs, while 11 demonstrated EMG abnormalities. All 21 patients with normal myelogram/CTs had normal SEPs. SEP improved electrodiagnostic sensitivity in patients with weakness or reflex changes as well as in those with sensory deficits only. SEP was less sensitive in patients in whom spinal stenosis was the only radiographic finding. MRI generally corresponded well with the results of myelogram/CT and SEP but overestimated the significance of disk bulges in some patients. SEP is useful in the electrodiagnostic evaluation of lumbosacral radiculopathy, particularly when EMG is nondiagnostic.     

Levetiracetam in patients with cortical myoclonus: a clinical and electrophysiological study.

Levetiracetam is a new antiepileptic agent that exerts antimyoclonic effects. We conducted an open-label trial to evaluate the effect of levetiracetam in chronic cortical myoclonus of diverse etiologies and to determine whether levetiracetam affects electrophysiological findings. Sixteen patients, aged between 19 and 72 years, with refractory, chronic, cortical myoclonus were recruited. We assessed myoclonus severity with the Unified Myoclonus Rating Scale (UMRS). The electrophysiological study comprised jerk-locked averaging, somatosensory evoked potentials (SEPs), and long loop reflex I. Levetiracetam was administered add-on at a starting dose of 500 mg twice per day up to the target dose of 50 mg/kg/day. Patients were reevaluated clinically and electrophysiologically 2 weeks after the titration phase. Fourteen patients completed the trial. Posttreatment UMRS scores showed an improvement of myoclonus in all cases. Pretreatment, 9 patients had "giant" SEPs. Posttreatment, the amplitude of these SEPs was reduced by more than 50% in 3 of 9 patients, and the mean N20-P25 amplitude was reduced significantly. Pre- and posttreatment SEP amplitude was not related to myoclonus severity or duration. Levetiracetam is a promising and a relatively easy-to-test antimyoclonic agent, which has the potential to improve significantly the patient's disability; however, its long-term efficacy should be verified in larger controlled studies.     

Lumbar and cortical somatosensory evoked potentials in rats with vitamin E deficiency.

Somatosensory evoked potentials (SEPs) from lumbar and cortical areas and electromyographic activity (EMG) were recorded in 40-42 week vitamin E deficient rats and in age matched controls. A significant increase in the latency (p less than 0.001) of the cortical SEP and a significant reduction in the lumbar to cortical conduction velocity (p less than 0.001) were observed in vitamin E deficient rats compared with controls. No significant differences were obtained in the latency of the lumbar SEP or in the peripheral conduction velocity from the ankle to lumbar region. All the vitamin E deficient rats had abnormal EMG findings (fibrillation potentials, positive sharp waves and polyphasic activity), whereas none of the controls showed any of these signs of dysfunction.