Central motor conduction in motor neuron disease

Central motor conduction was assessed in 13 patients with motor neuron disease and in 15 control subjects. All patients with motor neuron disease, even those without clinical pyramidal signs, had slowed central motor conduction, and in some the delays were asymmetrical. Evoked motor potentials represent a new and reliable method to detect physiological abnormalities of central motor pathways early in the course of motor neuron disease.     

Counting motor units in chronic motor neuropathies

The degree of motor unit loss can not be accurately quantified in chronic motor neuropathies with routine electrodiagnostic testing or with pathologic examination. We used motor unit number estimation (MUNE), which is a unique electrophysiologic method that can estimate the number of surviving motor units innervating a muscle, to study axonal loss in spinal muscular atrophy (SMA) and Charcot-Marie-Tooth (CMT) neuropathies. MUNE is based on the ratio of the maximal compound muscle action potential (CMAP) to the average surface-recorded motor unit potential (S-MUP). The hypothenar muscle group was studied in infant and older subjects with SMA, and the hypothenar and biceps-brachialis muscle groups were studied in adult CMT1A and CMT2 subjects. The multiple point stimulation MUNE technique was used in SMA subjects and the spike triggered averaging MUNE technique was used in CMT subjects. In SMA, motor unit loss was profound in types 1 and 2 subjects and more moderate in type 3 subjects. In CMT, motor unit loss was prominent in distal muscles in both CMT1A and 2 subjects, and present in proximal muscles in CMT2 subjects. MUNE is efficient in assessing the degree of motor unit loss in chronic motor neuropathies. SMA is considered to be a proximal muscle disorder, but loss was marked in distal muscles in all SMA types. In CMT1A, the demyelinating form, motor unit loss was marked in distal muscles, consistent with the idea that axonal loss and not slow conduction velocity is the important pathologic condition. The pattern of proximal motor unit loss differed between CMT1A and 2, suggesting differences in underlying axonal pathology.     

Volume of motor area predicts motor impulsivity

Impulsivity is a personality trait associated with many maladaptive behaviors. Trait impulsivity is typically divided into three different dimensions, including attentional impulsiveness, motor impulsiveness, and non‐planning impulsiveness. In the present study, we examined the neuroanatomical basis of the multidimensional impulsivity trait. Eighty‐four healthy participants were studied with structural magnetic resonance imaging. Multiple regression analyses revealed that the score of motor impulsiveness was negatively correlated with gray matter volumes of the right supplementary motor area and paracentral lobule. A machine‐learning‐based prediction analysis indicated that decreased gray matter volumes of the supplementary motor area and paracentral lobule strongly predicted the decrease in motor impulsiveness control. Our findings provide insights into the predictive role of motor brain structures in motor impulsivity and inhibition control.     

Multiple motor learning experiences enhance motor adaptability

Traditional motor learning theory emphasizes that skill learning is specific to the context and task performed. Recent data suggest, however, that subjects exposed to a variety of motor learning paradigms may be able to acquire general, transferable knowledge about skill learning processes. I tested this idea by having subjects learn five different motor tasks, three that were similar to each other and two that were not related. A group of experimental subjects first performed a joystick-aiming task requiring adaptation to three different visuomotor rotations, with a return to the null conditions between each exposure. They then performed the same joystick-aiming task but had to adapt to a change in display gain instead of rotation. Lastly, the subjects used the joystick-aiming task to learn a repeating sequence of movements. Two groups of control subjects performed the same number of trials, but learned only the gain change or the movement sequence. Experimental subjects showed generalization of learning across the three visuomotor rotations. Experimental subjects also exhibited transfer of learning ability to the gain change and the movement sequence, resulting in faster learning than that seen in the control subjects. However, transient perturbations affected the movements of the experimental subjects to a greater extent than those of the control subjects. These data demonstrate that humans can acquire a general enhancement in motor skill learning capacity through experience, but it comes with a cost. Although movement becomes more adaptable following multiple learning experiences, it also becomes less stable to external perturbation.     

Aberrant supplementary motor complex and limbic activity during motor preparation in motor conversion disorder

Conversion disorder (CD) is characterized by unexplained neurological symptoms presumed related to psychological issues. The main hypotheses to explain conversion paralysis, characterized by a lack of movement, include impairments in either motor intention or disruption of motor execution, and further, that hyperactive self-monitoring, limbic processing or top-down regulation from higher order frontal regions may interfere with motor execution. We have recently shown that CD with positive abnormal or excessive motor symptoms was associated with greater amygdala activity to arousing stimuli along with greater functional connectivity between the amygdala and supplementary motor area. Here we studied patients with such symptoms focusing on motor initiation. Subjects performed either an internally or externally generated 2-button action selection task in a functional MRI study. Eleven CD patients without major depression and 11 age- and gender-matched normal volunteers were assessed. During both internally and externally generated movement, conversion disorder patients relative to normal volunteers had lower left supplementary motor area (SMA) (implicated in motor initiation) and higher right amygdala, left anterior insula, and bilateral posterior cingulate activity (implicated in assigning emotional salience). These findings were confirmed in a subgroup analysis of patients with tremor symptoms. During internally versus externally generated action in CD patients, the left SMA had lower functional connectivity with bilateral dorsolateral prefrontal cortices. We propose a theory in which previously mapped conversion motor representations may in an arousing context hijack the voluntary action selection system, which is both hypoactive and functionally disconnected from prefrontal top-down regulation.     

Ocular motor function in motor neuron disease.

We studied ocular motor function in 34 patients with motor neuron disease (MND) and in 18 age-matched controls. This included the latency, accuracy, and amplitude-velocity relationships of saccades. We also examined ocular pursuit, the slow phases of optokinetic nystagmus, and the ability to suppress the vestibulo-ocular reflex (VOR) with visual fixation of a head-mounted target. Five of the subjects with MND had pronounced parkinsonian features on neurologic examination. The nonparkinsonian MND subjects had normal ocular motor function for all measures. Most subjects suppressed the VOR completely. The parkinsonian-MND patients had impairment of both saccadic and pursuit eye movements, and one parkinsonian-MND patient with poor pursuit was unable to suppress the VOR. We conclude that ocular motor function is generally spared in MND. The occasional appearance of ocular motor dysfunction probably reflects the incidence of secondary abnormalities such as parkinsonism.     

Cognitive motor processes: The role of motor imagery in the study of motor representations

Motor imagery is viewed as a window to cognitive motor processes and particularly to motor control. Mental simulation theory [Jeannerod, M., 2001. Neural simulation of action: a unifying mechanism for motor cognition. NeuroImage 14, 103–109] stresses that cognitive motor processes such as motor imagery and action observation share the same representations as motor execution. This article presents an overview of motor imagery studies in cognitive psychology and neuroscience that support and extend predictions from mental simulation theory. In general, behavioral data as well as fMRI and TMS data demonstrate that motor areas in the brain play an important role in motor imagery. After discussing results on a close overlap between mental and actual performance durations, the review focuses specifically on studies reporting an activation of primary motor cortex during motor imagery. This focus is extended to studies on motor imagery in patients. Motor imagery is also analyzed in more applied fields such as mental training procedures in patients and athletes. These findings support the notion that mental training procedures can be applied as a therapeutic tool in rehabilitation and in applications for power training.     

Spinal motor neurons and motor function in older adults

Journal of Neurology - This study examined the relation between lumbar spinal motor neuron (SMN) indices and motor function proximate to death in community-dwelling older adults. Older adults...     

Stimulation of motor tracts in motor neuron disease.

The muscle responses evoked by cortical and cervical stimulation in 11 patients with motor neuron disease were studied. The muscle potential in the abductor pollicis brevis, evoked by median nerve stimulation and the somatosensory potential evoked by wrist stimulation were also studied. In eight of 11 patients there was absence or increased central delay of the responses evoked by cortical stimulation. In four patients muscle responses on cervical stimulation and muscle action potentials on median nerve stimulation were also altered, indicating peripheral abnormalities. Somatosensory responses evoked by wrist stimulation were normal. Electrophysiological techniques are helpful in estimating the site of motor involvement in motor neuron disease.     

Understanding motor acts and motor intentions in Williams syndrome

Williams syndrome (WS) is a rare genetic disorder associated with unusually hyper-social demeanor and ease with strangers. These personality traits are accompanied by difficulties in social interactions, possibly related, at least in part, to a difficulty in understanding others' mental states. Studies on mentalizing capacities in individuals with WS have often led to contrasting results, some studies revealing specific impairments, others highlighting spared mentalizing capacities. So far, however, no study investigated the performance of individuals with WS in non-inferential understanding of others' motor intentions. In the present study we investigated this capacity by using a computer-based behavioral task using pictures of hand-object interactions. We asked individuals with WS first to describe what the other was doing (i.e. a task implying no kind of intention reading), and secondly, if successful in answering the first question, to describe the motor intention underlying the observed motor acts (i.e. why an act was being done, a task requiring non-inferential motor intention understanding). Results showed that individuals with WS made more errors in understanding what the other was doing (i.e. understanding a motor act) compared to both mental-age matched controls and chronological-age matched peers with typical development, while showing mental-age appropriate performance in understanding why an individual was acting (i.e. understanding a motor intention). These findings suggest novel perspectives for understanding impairments in social behavior in WS.     

Roles for perceived voluntary motor commands in motor control

Voluntary contractions of skeletal muscles are accompanied by centrally generated motor ‘commands’ that ultimately result in descending inputs to motoneurone pools. Perceived motor commands influence the sensations of muscle force and timing, and can be accurately directed to individual distal muscles in the absence of feedback. In addition, they may also influence respiratory sensations and cardiorespiratory responses to muscular contraction. Data on the neural mechanisms underlying these motor commands suggest that they may be be generated differently according to the particular function subserved within the motor system.     

Gammopathy with proximal motor axonopathy simulating motor neuron disease

We report a 38-year-old man with a pure motor syndrome and IgM gammopathy leading to flaccid quadriplegia. Improvement followed treatment with dexamethasone, cyclophosphamide, and plasmapheresis, but he died of pulmonary embolism. At autopsy, he had a proximal motor axonopathy with lymphocytic infiltration of ventral roots. Proximal motor neuropathy may masquerade as motor neuron disease. The association with gammopathy and response to treatment suggest that patients with motor neuron disease should be routinely screened for serum protein abnormalities.     

Initial motor impairment influences activation pattern of motor recovery

OBJECTIVES: We investigated the functional magnetic resonance imaging (fMRI) activation pattern of a motor task in patients with acute subcortical lesions to examine the relationship between activation pattern and recovery of motor impairment. METHODS: Five patients (one with subcortical infarction and four with thalamic hemorrhage) were examined using fMRI 1 month after the insult. Impairment was assessed by the Medical Research Council motor strength classification (MRC). One patient with severe motor deficits was also studied at 4 months when her motor deficits improved up to MRC grade 4. RESULTS: Three patients with relatively mild deficits (MRC grade 3 or 4) at their onsets, improved fully up to grade 5 within 1 month. FMRI performed at 1 month showed activation in the contralateral primary motor cortex and supplementary motor area (SMA), but no significant activation was seen on the ipsilateral unaffected side. Two patients with severe motor impairment (MRC grade 1) improved up to 3 and 4 of MRC at 1 month or later. They showed activation of the ipsilateral premotor area as well as contralateral primary motor cortex and SMA. One of them, whose severe motor deficit improved at 4 month, also showed activation of the ipsilateral postcentral gyrus and the activated area expanded longitudinally corresponding with her functional recovery. DISCUSSION: Our study demonstrates that the fMRI pattern varies according to functional recovery, suggesting the importance of the ipsilateral premotor area and postcentral gyrus especially for those patients with severe motor impairment initially.     

The reorganization of motor units in motor neuron disease

We studied 78 patients with motor neuron disease (MND) using concentric needle electromyography. Analysis on weak and maximal effort was performed using our own, fully automated, computer method, EMG-LAB. In addition to the conventional parameters of single motor unit action potentials (MUAPs) and interference pattern, new criteria were applied: the range of the acting motor units and the functional recruitment order. A total of 375 muscles of MND patients and 120 control muscles were investigated. The electromyographic data were analyzed separately in five groups of muscles, classified A, B, C, D, and E according to their clinical condition. Those results allowed us to discern six neurophysiological stages (N _{0, 1, 2, 3, 4, 5}) from the early to the most advanced phase. It has been confirmed that reinnervation in MND is adequate to compensate for the loss of over 50% of motor neurons but it is only a transitory phase in the morbid course. At stages N_0–5, the electrophysiological data reflect structural and functional integrity of the functioning motor units. Evaluation of not only single MUAPs but also of the full range of acting motor units and their recruitment order allowed a deeper look into the underlying pathophysiological mechanisms. © 1997 John Wiley & Sons, Inc. Muscle Nerve, 20, 306–315, 1997.     

Suppression of ipsilateral motor cortex facilitates motor skill learning

The primary motor cortex (M1) plays a critical role in early aspects of motor skill learning. Given the notion of inter-hemispheric competition, unilateral disruption of M1 may increase excitability of the unaffected motor cortex and thus improve motor learning with the ipsilateral hand. We applied slow-frequency repetitive transcranial magnetic stimulation (rTMS) before the initiation of practice of a simple motor skill. Participants were randomly divided into three stimulation groups: (i) ipsilateral M1; (ii) contralateral M1; and (iii) Cz (control site). The mean execution time and error rate were recorded in four sessions distributed over 2 days. Disruption of M1 with rTMS slowed down skill acquisition with the contralateral hand, albeit non significantly, but paradoxically enhanced learning with the ipsilateral hand. This was evidenced by a significant decrease of execution time at the end of day 1 in the group that received rTMS over the ipsilateral M1 compared with both control groups (Cz and contralateral M1 stimulation). This supports the notion of inter-hemispheric competition and provides novel insights that may be applicable to neurorehabilitation.     

Ipsilateral involvement of primary motor cortex during motor imagery

To investigate whether motor imagery involves ipsilateral cortical regions, we studied haemodynamic changes in portions of the motor cortex of 14 right-handed volunteers during actual motor performance (MP) and kinesthetic motor imagery (MI) of simple sequences of unilateral left or right finger movements, using functional magnetic resonance imaging (fMRI). Increases in mean normalized fMRI signal intensities over values obtained during the control (visual imagery) task were found during both MP and MI in the posterior part of the precentral gyrus and supplementary motor area, both on the contralateral and ipsilateral hemispheres. In the left lateral premotor cortex, fMRI signals were increased during imagery of either left or right finger movements. Ipsilateral cortical clusters displaying fMRI signal changes during both MP and MI were identified by correlation analyses in 10 out of 14 subjects; their extent was larger in the left hemisphere. A larger cortical population involved during both contralateral MP and MI was found in all subjects. The overall spatial extent of both the contralateral and the ipsilateral MP + MI clusters was approximately 90% of the whole cortical volume activated during MP. These results suggest that overlapping neural networks in motor and premotor cortex of the contralateral and ipsilateral hemispheres are involved during imagery and execution of simple motor tasks.     

Preferential reinnervation of motor nerves by regenerating motor axons

Regeneration of axons into inappropriate distal nerve branches may adversely affect functional recovery after peripheral nerve suture. The degree to which motor axons reinnervate sensory nerves, and vice versa, has not been determined. In these experiments, HRP is used to quantify the sensory and motor neurons that reinnervate sensory and motor branches of the rat femoral nerve after proximal severance and repair. Motoneurons preferentially reinnervate the motor branch in juveniles and adults, even if the repair is intentionally misaligned or a gap is imposed between proximal and distal stumps. A specific interaction thus occurs between regenerating motor axons and the Schwann cell tubes that lead to the motor branch. This interaction is independent of mechanical axon alignment.     

Effects of motor imagery are dependent on motor strategies

To investigate whether the facilitatory effects of motor imagery (MI) are dependent on motor strategies that vary with posture, we used transcranial magnetic stimulation to examine the effects of two forearm positions on motor-evoked potentials during an MI of index-finger abduction. MI-enhanced motor-evoked potentials of the first dorsal interosseous (prime mover) muscle in the forearm prone position were larger than those in the forearm neutral position. The opposite effects were seen in the extensor carpi radialis (synergist) muscle. These effects correspond to the different electromyography activities in the muscles when performing the actual movements in these two forearm positions. It is suggested that MI reflects different motor strategies in the contribution of agonist and synergist muscles towards a motor task.     

Single motor unit H-reflex in motor neuron disorders.

The latency fluctuation of single motor unit potentials (MUPH) in the H-reflex is greater than the latency fluctuation of MUPs in the direct (MUPM) and recurrent (MUPF) responses. This has been attributed to the variability in the impulse generation at the site of nerve stimulation, and to the variation in the synaptic delay at the anterior horn cell. We studied the latency fluctuation of single motor unit H-reflex in patients with motor neuron disorders (MND) in comparison with normal subjects. The mean jitter of the H-reflex was 264.3 +/- 17.8 microseconds (mean +/- SEM) in 30 MUPH recorded from 10 patients with ALS, 302.7 +/- 25.2 microseconds in 16 MUPH from 6 patients with chronic motor neuron diseases, as compared with 137.4 +/- 7.3 microseconds in 34 MUPH recorded from 10 normal subjects. This difference, which persisted even after the correction for the latency variation of MUPM, cannot be explained on the basis of an enhanced reciprocal inhibition. Thus, the increased latency fluctuation of the single motor unit H-reflex in patients with MND may reflect changes in the motoneuron pool excitability that may be secondary to altered intrinsic electrophysiological properties of motoneurons, or to an abnormal temporal and spatial summation of synaptic inputs on motoneurons.