Movement-related cortical potentials in patients with Machado-Joseph disease.

ObjectiveMovement-related cortical potentials (MRCP; nomenclature of MRCP components according to Shibasaki and Hallett (Shibasaki H, Hallett M. What is the Bereitschaftspotential? Clin Neurophysiol 2006;117:2341–56) were studied in patients with Machado–Joseph disease (MJD) to elucidate the pathophysiology of voluntary movement.MethodsWe studied nine genetically proven MJD patients and eight age-matched healthy subjects. Multi-channel electroencephalogram (EEG) recordings were obtained during self-paced fast extensions of the wrist. EEG epochs were time-locked to electromyography (EMG) onset or offset of the voluntary EMG burst and averaged.ResultsIn the MJD patients, the early Bereitschaftspotential (early BP, −1500 to −500 ms) was not affected but the late BP was reduced over the central midline area and contralaterally to the movement side. The amplitude of the fpMP, a post-movement MRCP component, was also reduced. In addition, the offset cortical potential in the first 500 ms after EMG offset (Moff + 500) was attenuated bilaterally over a wide cortical area.ConclusionsFindings suggest that cortical activations associated with the initiation and termination of a voluntary movement are impaired in MJD patients.SignificanceAbnormalities of pre- and post-movement MRCP components provide researchers with pathophysiological insight into voluntary motor dysfunction in MJD.     

Movement-related cortical potentials associated with progressive muscle fatigue in a grasping task.

OBJECTIVE: The present research was aimed to further address the general empirical question regarding the behavioral and neurophysiological indices and mechanisms that contribute to and/or compensate for muscle fatigue. In particular, we examined isometric force production, EMG, and EEG correlates of progressive muscle fatigue while subjects performed a grasping task. METHODS: Six neurologically healthy subjects were instructed to produce and maintain 70% of maximum voluntary contraction (MVC) for a total of 5 s in a sequence of 120 trials using a specially designed grip dynamometer. Three components of movement-related potentials (Bereitschaftspotential, BP, Motor potential, MP, and Movement-monitoring potential, MMP) were extracted from continuous EEG records and analyzed with reference to behavioral indicators of muscle fatigue. RESULTS: Experimental manipulations induced muscle fatigue that was demonstrated by decreases in both MVC values and mean force levels produced concomitant to increases in EMG root mean square (RMS) amplitude with respect to baseline levels, and EMG slope. EEG data revealed a significant increase in MP amplitude at precentral (Cz and FCz) and contralateral (C3) electrode sites, and increases in BP amplitude at precentral (Cz and FCz) electrode sites. CONCLUSIONS: The increases in EMG amplitude, EMG slope, and MP amplitudes suggest a possible link between the control signal originating in the motor cortex and activity level of the alpha-motoneuron pool as a function of progressive muscle fatigue. Overall, the data demonstrate that progressive muscle fatigue induced a systematic increase in the electrocortical activation over the supplementary motor and contralateral sensorimotor areas as reflected in the amplitude of movement-related EEG potentials.     

Movement-related cortical potentials in myotonic dystrophy.

OBJECTIVE: To investigate a possible deficit of the voluntary movement mechanism within the central nervous system (CNS) in patients with myotonic dystrophy (MyD). METHODS: Movement-related cortical potentials preceding voluntary extension of the right middle and index fingers were studied in 9 patients with MyD and compared with those in 11 age-matched healthy subjects and 9 age-matched patients with other neuromuscular disorders (NMDs). RESULTS: The amplitudes of Bereitschaftspotential was smaller in MyD patients than in age-matched controls and age-matched patients with other NMDs although there was no statistically significant difference. The amplitude of negative slope was significantly smaller in MyD patients than in age-matched controls and age-matched patients with other NMDs. Clinical findings such as age, disease duration, degree of motor impairment and cognitive function had no effect on the individual electrophysiological parameters. CONCLUSIONS: The present results suggest that subclinical abnormalities exist in CNS function associated with motor preparation and execution, which is independent of muscle weakness.     

Movement-related cortical potentials in writer's cramp

Movement-related cortical potentials in response to simple, self-paced, brisk index finger abduction movements were recorded in patients with simple and complex writer's cramp and compared with those of age-matched control subjects. Analysis of the movement-related cortical potential waveforms showed that the Bereitschaftspotential, the peak of the negative slope, and the frontal peak of the motor potential did not differ in the two groups, except for the average amplitude of the early part of the negative-slope peak, which was decreased in the patient group during the interval of 300 to 200 msec prior to electromyographic onset. This finding was restricted to the electrodes overlying the contralateral and midline central electrodes. Movement-related cortical potentials from patients and control subjects could be equally accounted for by a four-dipole source model with sources located in the contralateral and ipsilateral sensorimotor regions and the supplementary motor area. There was a trend for a reduction in the strength of the sensorimotor sources active during the premotor period in the patient group, but the difference did not reach a significant level for any individual source. No differences were found between the movement-related cortical potentials elicited by movements of the affected and unaffected hand, or between those of patients with simple or complex hand cramps. This result suggests a deficiency of contralateral motor cortex activation just prior to the initiation of voluntary movements in patients with focal dystonia.     

Movement-related cortical potentials in patients with cerebellar degeneration

We studied the topographic distribution of scalp-recorded, averaged movement-related cortical potentials occurring immediately before and after the onset of voluntary movements in six patients with cerebellar degenerative disease. We placed 26 electrodes on the scalp overlying the sensorimotor area and recorded cortical potentials related to abduction of the index finger. The amplitudes and latencies of the potentials were normal in all patients except two, in whom the negative slope (NS') was absent. All patients had an abnormal topographic pattern of potentials compared with normal subjects. The initial slope of motor potential (isMP), which was focal and contralateral in the normal subjects, was diffuse and bilateral in the patients. The topography of the frontal peak of motor potential (fpMP) was more posterior in the patients than in normal subjects. The patterns found in this preliminary study indicate a derangement of sensorimotor cortex activity in voluntary movement as a consequence of cerebellar dysfunction.     

Movement-related cortical potentials in aged subjects]

Cortical potentials related to freely-executed voluntary wrist flexion (MRPs) were studied in 35 subjects aged 23-80 years. The characteristics of the MPRs in aged subjects were determined in comparison data from 14 young subjects aged 23-40 years. The analysis concerned 3 components of the MRPs: the slow negative shifts (NS1 and NS2) before the movement onset and the motor potential (MP). In the aged subject, the latencies measured at Cz show a significant lengthening of the NS1 and of the duration of NS2 (NS' of Shibasaki et al, 1980). The mean amplitude of the NS1 peak at Cz is decreased, and those of N1 (the negative peak before the movement) and MP are not significantly different from those of the young subjects. The NS2 component in the aged subject (between NS1 and N1) is thus increased. In contrast to the young subjects, who present a predominance of N1 and MP amplitudes of the contralateral motor cortex over the ipsilateral cortex, the aged subjects lose lateralization of these components. Recording of MRPs with subdural electrodes (Neshige et al, 1988) shows taht NS1 results from the activity of the supplementary motor area and from the ipsi-contralateral primary motor cortex. The increase in NS2 might be interpreted as an expression of activity coming from other structures to compensate for the reduction in NS1 in the aged subject and to maintain the level of the motor potential MP.     

Movement-related cortical potentials in primary lateral sclerosis

OBJECTIVE: Some patients with primary lateral sclerosis (PLS) have a clinical course suggestive of a length-dependent dying-back of corticospinal axons. We measured movement-related cortical potentials (MRCPs) in these patients to determine whether cortical functions that are generated through short, intracortical connections were preserved when functions conducted by longer corticospinal projections were impaired. METHODS: An electroencephalogram was recorded from scalp electrodes of 10 PLS patients and 7 age-matched healthy control subjects as they made individual finger-tap movements on a keypad. MRCPs were derived from back-averaging the electroencephalogram to the movement. RESULTS: MRCPs produced by finger taps were markedly reduced in PLS patients, including components generated by premotor areas of the cortex as well as the primary motor cortex. In contrast, the beta-band event-related desynchronization from the motor cortex was preserved. INTERPRETATION: These findings suggest that impairment in PLS is not limited to the distal axons of corticospinal neurons, but also affects neurons within the primary motor cortex and premotor cortical areas. The loss of the MRCP may serve as a useful marker of upper motor neuron dysfunction. Preservation of event-related desynchronization suggests that the cells of origin differ from the large pyramidal cells that generate the MRCP.     

Movement-related cortical potentials before jaw excursions in oromandibular dystonia.

Oromandibular dystonia is a neurological disorder characterized by involuntary contraction of masticatory and/or tongue muscles. Cortical negative shifts preceding voluntary movements called "movement-related cortical potentials" (MRCPs) reflect a central motor control process. Reduced amplitude of MRCPs has been reported in other types of dystonia. To elucidate whether the abnormality is observed also in oromandibular dystonia, we compared MRCPs associated with mandibular movements in 6 patients with this condition and in 8 normal subjects. Electroencephalograms (EEGs) were recorded from 11 electrodes, and electromyograms (EMGs) were recorded from the masseter muscle and the suprahyoid muscles. The subjects were asked to repeat mouth opening, closing, and left and right lateral mandibular excursions. MRCPs were obtained by averaging the EEG using the EMG onset as the trigger signal. In the patient group, MRCP amplitudes over central and parietal areas for mouth opening and lateral movements were significantly reduced compared to normal subjects. In normal controls, the MRCPs at mouth opening and closing were symmetrically distributed, whereas those at lateral movements showed predominance over the hemisphere ipsilateral to the direction of the movement. This laterality was lost in the patient group. These results suggest impaired cortical preparatory process for jaw movements in oromandibular dystonia.     

Movement-related potentials and control of associated movements

Previous studies have shown a relationship of the readiness potential (RP) preceding a motor act to motor control, as indexed by eye movement (EM). Greater EM and, therefore, less motor control was associated with increased positivity in preresponse RP components. It was hypothesized that these positive components may reflect processes involved in the inhibition of extraneous or associated movement during the performance of a motor act, especially in younger subjects with less motor development. We developed a finger lift task for detecting irrelevant associated movements (AM) from the responding hand and the nonresponding contralateral hand. During each target finger lift, small movements of the other nontarget fingers from the target hand and the contralateral hand were considered movements that should have been inhibited. Trials for each subject were divided into two bins: associated movement (AM) trials which had movement of target plus nontarget fingers, and trials with only target finger movement detected (NAM). Difference waveforms indicated a positive-going shift on trials with discrete target finger movements (NAM). Age and RP positivity at ipsilateral and posterior regions were significantly correlated. We suggest that, on trials on which associated movements are successfully inhibited, the negativity of the RP is confounded by an overlapping slow positivity. The positivity may be related to the effort needed to inhibit associated movements in order to perform a sharper and more discrete response. This relationship is a function of motor control and, indirectly, of age.     

Comparative study of cerebral cortical potentials associated with voluntary movements in monkey and man

Eight monkeys (Macaca mulatta) were taught to squeeze and release a handgrip. The movement simulated the brisk squeeze of a hand dynamometer performed by 7 human subjects. Monkey. During the performance of the voluntary movements, slow cortical potentials (motor potentials or MPs) were studied with monopolar, surface bipolar, transcortical and intracortical recordings. A survey of the dorsal expanse of cerebral cortex showed that the contralateral motor hand area, somatosensory hand area and area 6 adjacent to the supplementary motor area became active with movement. MPs also were seen in the motor and somatosensory cortex medial to the hand area, but we concluded that those potentials were probably related to adventitious movements in the arm and leg. That area 6 became active with movement was further verified with extracellular unit recording; the behavior of area 6 units was compared with that recorded from units in the motor hand area. Using simultaneous transcortical recordings a sequence of cortical activation was observed in those areas generating an MP. The motor hand area became active first, followed in turn by area 6 and the somatosensory hand area. The monosynaptic cortico-cortical connections of the motor hand area were studied with autoradiographic and horseradish peroxidase techniques and compared to the distribution of the MP. The hand area demonstrated reciprocal connectivity with portions of the somatosensory hand area, the supplementary motor area in area 6 and the cortex adjacent to the intraparietal sulcus. The distribution of the MPs correlated with the connectivity to the supplementary motor and somatosensory areas. Our physiologic studies did not adequately investigate the area adjacent to the intraparietal sulcus. Man. Motor potentials were studied using surface bipolar recordings with closely spaced electrodes (inter-electrode distances 1 cm or 2 cm). Recordings were made directly from the cortex in one subject studied under local anesthesia during an operation for epilepsy, and epidurally in 6 subjects in whom epidural electrode arrays had been inserted for the purpose of localizing an epileptogenic focus. Similar to the findings in the animals, MPs were recorded from the contralateral motor and somatosensory hand area with activity in motor cortex appearing first; area 6 just anterior to the motor hand area probably also generated a response. In addition, a locally generated potential not seen in monkey was recorded anterior to area 6. This difference in response distribution is viewed as possibly relating to the different significance which the seemingly comparable hand movements have for the animal and human subjects. No response was seen in motor and somatosensory hand area with ipsilateral movements. We have no information for the anteriorly recorded response with ipsilateral movement.     

Movement-related slow potentials. I. A contrast between finger and foot movements in right-handed subjects

Movement-related potentials ( MRPs ) preceding a finger flexion and a plantar flexion of the foot on either side were compared over the frontal, central and parietal areas of both hemispheres. MRP amplitudes were larger preceding foot than preceding finger movements. In the first case their onset was earlier and their presence in the frontal area was more marked. Prior to a finger flexion amplitudes over the hemisphere contralateral to the movement side were larger than those recorded over the ipsilateral hemisphere. On the contrary, prior to a plantar flexion of the foot, amplitudes were larger over the hemisphere ipsilateral to the movement. These findings point to differently localized sources of the MRPs in the two cases. In other experiments larger amplitudes preceding foot movements were found near the midline. It is suggested that the ipsilateral preponderance prior to foot movements is caused by a contralateral source in the depth near the longitudinal fissure. The dipoles are presumably directed obliquely to the median plane. The ipsilateral preponderance is present both prior to and following the plantar flexion. This suggests comparable directions of the dipoles in the motor and somatosensory areas.     

Movement-related cortical potentials preceding repetitive and random-choice hand movements in parkinson's disease

The movement-related cortical electroencephalographic potential was recorded from scalp electrodes in 8 patients with idiopathic Parkinson's disease studied at least 12 hours after withdrawal of their normal drug therapy, and compared with the results from a group of 8 age-matched control subjects. Two types of self-paced voluntary arm movements were examined: repetitive forward movement of a joystick, and random-choice movements of the same joystick in which subjects had to choose freely the direction in which they were to move the stick (forward, backward, left, or right). In normal subjects, the movement-related cortical potential was larger prior to random-choice movements, whereas in the patients, the amplitude was the same in both tasks. The implication is that processes involved in self-selection of movement are abnormal in Parkinson's disease. This may contribute to the difficulty that patients have in initiating voluntary movement in the absence of any external cues.     

Abnormal cortical mechanisms in voluntary muscle relaxation in de novo parkinsonian patients.

This study aimed at elucidating how the cortical mechanism underlying the preparation and the postmovement phase of voluntary hand muscle relaxation is affected in Parkinson's disease. Event-related mu and beta (de)synchronization (ERD/S) related to voluntary muscle contraction and relaxation were recorded in 16 untreated, akineto-rigid, predominantly hemiparkinsonian patients. The results were compared with data from 10 age-matched, healthy subjects. In the muscle relaxation task, the subject held the wrist in an extended position and then let the hand drop by voluntarily relaxing wrist extensor contraction, i.e., without any overt, associated muscle contraction. In the muscle contraction task, subjects performed a self-initiated brief wrist extension. A same pattern of ERD/S was observed in control subjects and parkinsonian patients performing the motor tasks with their less affected limb. In contrast, related to voluntary relaxation performed with the more affected limb, a delayed mu and beta ERD and a disappearance of beta ERS were revealed. These results demonstrate that the pattern of cortical oscillatory activity in a relaxation task is abnormal in parkinsonian patients. The authors suggest that this may be due to anomalous activity in inhibitory motor cortical systems and impaired sensorimotor integration of afferent inputs from muscle and joint receptors.     

Oscillatory cortical activity related to voluntary muscle relaxation: influence of normal aging.

OBJECTIVE: In this study we aimed to investigate if there are age-related differences in cortical oscillatory activity induced by self-paced muscular pure relaxation in comparison with muscle contraction as reference movement. METHODS: Event-related (de)synchronization (ERD/ERS) have been recorded related to voluntary muscle contraction and relaxation in 10 young and 10 elderly right-handed healthy subjects. The muscle relaxation task consisted in a voluntary relaxation of maintained wrist extension without any overt, associated muscle contraction. The muscle contraction task corresponded to a self-initiated brief wrist extension. RESULTS: In elderly subjects compared to young ones, mu and beta ERD preceding muscular relaxation was more widespread, beginning significantly earlier over contralateral frontocentral and parietocentral regions (p<0.05) as well as over ipsilateral regions (p<0.05). The beta synchronization was significantly attenuated (p<0.05). CONCLUSIONS: These results suggest an alteration of inhibitory motor systems and an altered post-movement somesthetic inputs processing with normal aging. These alterations were accompanied by compensatory mechanisms. SIGNIFICANCE: These age-related alterations during different phases of muscle relaxation could participate to explain global sensorimotor slowing observed with normal aging.     

Movement-related cortical potentials: their relationship to the laterality, complexity and learning of a movement

Movement-related cortical potentials (MRCPs) to self-paced unilateral movements of different laterality, complexity and practice level were recorded from 14 healthy subjects using the EMG onset as the trigger. The amplitudes at certain time points and slopes of linear regression lines fitted to 3 main shifts have been evaluated. In the early phase of MRCP the potential and slope values were symmetrically distributed around the midline maximum, which indicates that this part of MRCP can not originate from the primary motor area, which is in a contralateral relation with the movement, but from secondary motor areas (SMA and premotor areas). The changes in the voltage levels and slopes of this phase due to the changes in laterality, complexity and practice level of the movement show the relation of this activity with the abstract characteristics of the movement. The decrease of voltages and slope values in the later phases of MRCP in the complex task, which is replaced by higher voltages and slopes after a certain learning period was evaluated as a result of inhibition of associative movements via reafferent feedback signals occurring often in the first stages of learning period.     

Expectancy-related cerebral potentials associated with voluntary time estimation and omitted stimulus

Expectancy-related and nonexpectancy-related cerebral potentials associated with stimuli and omitted stimuli were recorded in 7 normal subjects. The stimuli were constantly delivered to the right median nerve and the interstimulus interval was set at 7 seconds. When the subject counted to estimate the interstimulus interval correctly, a slow negative deflection appeared about one second prior to both the stimuli and the omitted stimuli. In the case of the omitted stimulus, this expectancy-related negative potential (ENP) returned to the base line after several hundred msec. When the stimuli were delivered, the amplitude of the P300 was much higher when the subject was paying attention to the stimuli than when he was not. The scalp distribution of the ENP was rather anterior to the P300. No ENP appeared when the subject was not paying attention to the stimuli or the omitted stimuli, or when the stimuli were delivered at a random rate.     

Movement-related potentials accompanying unilateral and bilateral finger movements with different inertial loads.

The present study was aimed at investigating the effect of inertial loading on movement-related potentials (MRPs) recorded from the scalps of normal subjects while performing finger movements. Two experiments were performed. Experiment 1. MRPs preceding and accompanying the execution of voluntary, unilateral finger movements were investigated in 8 subjects under the 3 experimental conditions of: no inertial load, small inertial load (250 g), and large inertial load (400 g). A significant effect of the inertial load on Bereitschaftspotential (BP) amplitude was observed for the 100 msec period preceding movement onset (BP -100 to 0) at precentral electrode sites and following movement onset (N0 to 100) at both precentral and parietal electrode sites. Pairwise comparisons revealed that significant effects were due to differences between the loading and non-loading conditions and not for different amounts of loading. No significant differences were observed for BP onset or early BP amplitudes, indicating that scalp negativity immediately prior to, and during, movement onset is primarily influenced by conditions of inertial loading. Experiment 2. This experiment examined the effect of inertial loading on MRPs for bilateral, simultaneous voluntary finger movements in 10 subjects under conditions of: no inertial load, inertial load applied separately to the left and right fingers, and with identical inertial loads applied to both fingers. No significant effect of inertial load on MRP amplitude was observed. These results are contrasted with those of experiment 1 which show significant effects of inertial loading for unilateral movements and are interpreted in terms of the hypothesis that bilateral movement organization involves 'higher' aspects of motor control than those reflecting adjustment to conditions of inertial loading.     

Movement-related cortical potential in patients with cerebellar dentate degeneration]

We reported movement-related cortical potentials (MRCPs) in 11 patients with lesion of the dentate nucleus (Machado-Joseph disease (MJD) 7 cases, dentato-rubro-pallido-luysian atrophy (DRPLA)1, myoclonus epilepsy associated with ragged-red fibers (MERRF)1, dyssynergia cerebellaris myoclonica (DCM) 2), and compared with those of 7 cases of multiple system atrophy (MSA) who were postulated to have mild dentate lesions (striato-nigral degeneration 2 cases, Shy-Drager syndrome 2, sporadic olivo-ponto-cerebellar atrophy 3), and 7 control subjects without any neurological findings. Further we classified the diseases into the following two groups based on the lesion of the dentate nucleus. One was MJD group that had normal or slightly abnormal electroencephalogram (EEG), and the other was DN group (DRPLA, MERRF, DCM) that had markedly abnormal EEG. One of the main findings from this study was smaller slope of the Ns' in the MJD and DN group and normal slope of BP. There was no significant difference in the slope of Ns' between MJD patients and DN patients. This result shows EEG abnormalities have no influence on MRCP recordings. These results suggest that Ns' component may reflect the function in the cerebellar dentate nucleus, and that MRCP is a useful diagnostic method in patients with cerebellar ataxia.