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Biomedical subjects

D Cros

Publications and source records attributed to D Cros.

At least 37 records · Page 2Linked to original sources
PubMed1994

There are no neurophysiologic features characteristic of "axonal" Guillain-Barré syndrome.

Classical views hold Guillain-Barré syndrome (GBS) as a primary inflammatory-demyelinating neuropathy in which secondary axonal degeneration may occur, particularly when inflammatory lesions are severe. Feasby and colleagues proposed that primary axonal degeneration can also cause GBS characterized by inexcitable motor nerves and poor outcome. This hypothesis rests largely on the results of a single autopsy in which no inflammation or demyelination were found. Using an illustrative case report confirming earlier studies, we point out that inexcitable motor nerves (or low amplitude compound muscle action potentials [CMAPs]) are of ambiguous significance and may reflect distal demyelination, causing conduction block between distal stimulation sites and target muscles, a pattern not uncommon in GBS. Recovery from such lesions may occur within weeks with restoration of CMAP amplitudes. The recognition of a yet unproven axonal variant of GBS cannot be based solely on documentation of inexcitable motor nerves in the context of rapidly developing weakness.

Action Potentials↗
PubMed1994

Quantitative analysis of the compound muscle action potential in early acute inflammatory demyelinating polyneuropathy.

We quantitated the size and configuration of compound muscle action potentials (CMAPs) in 266 nerves (66 median, 67 ulnar, 71 tibial and 62 peroneal) of 72 patients with acute inflammatory demyelinating polyneuropathy (AIDP) initially studied within 19 days of symptom onset. Results were compared with criteria for CMAP abnormalities, including criteria for abnormal negative peak duration and desynchronisation, derived from a control population of 50 median, ulnar, tibial and peroneal nerves. Other motor conduction abnormalities including minimal F response latency were also examined. We also analysed patterns of CMAP abnormality, peak disability and outcome for AIDP patients who had at least 3 motor nerves evaluated at first electrophysiologic study. Amongst AIDP nerves, low amplitude of the distal CMAP, usually with prolonged distal latency, was much more common than an abnormal fall in CMAP amplitude between stimulus sites. Using our CMAP criteria more than half of these low amplitude distal responses showed prolonged negative peak duration of desynchronisation or both, consistent with demyelination. Of the 47 AIDP patients who had 3 or more nerves initially studied, 37 (78.7%) had at least 1 motor nerve with a distal CMAP showing evidence of temporal dispersion. In addition, those with at least 75% of motor nerves showing a pattern of low amplitude of the distal CMAP without a further significant fall in amplitude between stimulus sites had greater peak disability and a poorer outcome. Assessment of temporal dispersion of the distal CMAP should be included in electrophysiologic criteria for acute demyelination. In addition, for some patients with AIDP patterns of CMAP amplitude abnormality amongst motor nerves are present early in the illness and may provide prognostic information.

Action Potentials↗
PubMed1994

Quantitative studies of F responses in Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy.

We examined F wave mean and minimum latency, mean and maximum amplitude, duration, persistence and chronodispersion in 241 nerves from 78 patients with Guillain-Barré syndrome (GBS) and 162 nerves from 43 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Results were compared with normal criteria derived from 72 median, 73 ulnar and 73 tibial control nerves, to determine the relative diagnostic sensitivity of the various F wave parameters. F wave abnormalities were found in 92% and 95% of nerves of patients with GBS and CIDP respectively. Absence of F responses or prolongation of minimum and mean latency were the most frequent abnormalities in both groups. Forty-five (11.2%) nerves overall had absent F responses with normal compound muscle action potential (CMAP) amplitudes and no significant fall between stimulus sites, consistent with isolated proximal conduction block. Forty-four nerves (23.7% of nerves in which F waves were present) fulfilled minimum F latency criteria for acquired demyelination . Eighty-one (20.1%) nerves had normal conventional motor nerve conduction studies and abnormal F responses, not all of which were identified by assessing only F absence or minimum latency. Severity of F wave abnormalities did not correlate with clinical outcome. Our findings confirm the high frequency of proximal nerve lesions in early GBS and CIDP, not all of which are associated with distal motor conduction abnormalities, and suggest that assessment of multiple F wave parameters, in particular chronodispersion, mean latency and mean amplitude (in addition to absence and minimum latency), increases the yield of F wave studies.

Adult↗
PubMed1993

Cortical and spinal motor excitability during the transcranial magnetic stimulation silent period in humans.

We investigated the electromyographic silent period in abductor pollicis brevis (APB) and flexor carpi radialis muscles following transcranial magnetic stimulation of human motor cortex. In APB, we measured cortical stimulation silent period (CSSP) duration as a function of stimulus intensity, motor-evoked potential (MEP) amplitude and muscle twitch force. We used peri-stimulus-time histograms to study the effect of cortical stimulation on single-motor unit firing patterns. We compared F-waves, H-reflexes and magnetic MEPs elicited during the CSSP to control responses elicited at rest and during voluntary contraction. CSSP duration depended on the intensity of cortical stimulation. However, we found no relationship between CSSP duration and MEP amplitude or muscle twitch force, thus the CSSP is not dependent solely on Renshaw cell inhibition or on changes in Ia and Ib afferent activity following the cortically induced muscle twitch. At low intensities of stimulation, the interval to resumption of motor unit firing following the peak in the peri-stimulus-time histogram corresponding to MEP latency sometimes exceeded that which could be accounted for by the motor unit's firing rate prior to the stimulus, suggesting that synchronization of motor unit firing by cortical stimulation cannot account for the CSSP. We found brief inhibition of F-waves during the CSSP in some subjects, reflecting activation of inhibitory corticospinal projections or segmental effects. In contrast, we observed longer inhibition of H-reflexes during the CSSP in all subjects, perhaps resulting from presynaptic inhibition of Ia afferents. Magnetic MEPs also were inhibited during the CSSP, suggesting inhibition of cortical elements by transcranial magnetic stimulation.

Adult↗
PubMed1993

Variability of motor potentials evoked by transcranial magnetic stimulation.

We studied the effect of stimulus intensity, coil size, mental alertness and prestimulus muscle contraction on the variability of motor evoked potentials (MEPs) produced by magnetic cortical stimulation (MCS). In 5 healthy subjects we delivered MCS either with a circular coil centered at the vertex or a figure-8 coil centered over the motor cortex hand area, recording from first dorsal interosseous. With the subject at rest or exerting 5% maximum voluntary contraction, 30 consecutive stimuli were given at 4 stimulus intensities (SIs) in 10% increments above resting motor threshold. Concurrent mental arithmetic constituted mental alertness. Spectral analysis was performed on data from 300 consecutive stimuli. The variability of MEP response size was inversely related to stimulus intensity, prestimulus voluntary muscle contraction, the recruitment of motoneurons and the size of the field generated by the magnetic coil. The MEP variability was larger than and not correlated with the variability of the H-reflex. Fast Fourier transformation and cross-correlation analysis did not identify a consistent dominant frequency, suggesting that the variability in MEP size is essentially random. We suggest that the variability in MEP response is caused by constant, rapid, spontaneous fluctuations in corticospinal and segmental motoneuron excitability levels. Any maneuver that raises this level or increases the probability of motoneuron firing will decrease MEP variability.

Adult↗
PubMed1993

Facilitation of magnetic motor evoked potentials during the cortical stimulation silent period.

We investigated the relationship between stimulus intensity and magnetic motor evoked potentials (MEPs) elicited 100 msec after a conditioning stimulus that was 25% of stimulator output above resting motor threshold (RMT) during tonic contraction of abductor pollicis brevis. In five subjects, MEPs elicited with stimuli less than 25% above RMT were inhibited during the EMG cortical stimulation silent period (CSSP) produced by the conditioning stimulus, relative to MEPs elicited with the test stimulus given at rest. However, increasing the intensity of the test stimulus increased the amplitude of MEPs elicited during the CSSP relative to MEPs elicited at rest, such that MEPs elicited with stimuli 30 to 45% above RMT were facilitated during the CSSP. Increasing the intensity of the test stimulus also increased the amplitude of MEPs elicited with paired stimulation at rest, and caused facilitation in one subject. Since facilitation of MEPs was never accompanied by shortening of MEP latency, our observations point to supraspinal facilitory mechanisms. We suggest that facilitation of MEPs during the CSSP reflects temporal and spatial summation of conditioning and test stimuli.

Adult↗
PubMed1992

Motor inhibition and excitation are independent effects of magnetic cortical stimulation.

We administered magnetic cortical stimulation (MCS) during voluntary contraction of intrinsic hand muscles to 8 patients with motor neuron disease (MND), 5 patients with pure lower motor neuron syndromes (LMN), a patient with severe subacute sensory neuropathy (SSN), and 10 healthy volunteers. Patients with MND had clinical evidence of upper MND and elevated thresholds for (3 patients) or absence of (5 patients) motor evoked potentials (MEPs). MCS during sustained contraction inhibited electromyographic activity in 6 of 8 patients with MND, without preceding MEPs. MCS had no effect on the electromyogram (EMG) of the other 2 patients with MND. In normal subjects and patients with LMN, inhibition of EMG was never seen without a preceding MEP, regardless of stimulus intensity. In the patient with SSN, MCS elicited normal MEPs and inhibited the EMG in a pattern similar to normal subjects, whereas supramaximal electrical stimulation of median and ulnar nerves failed to inhibit the EMG despite normal M and F responses. Our findings indicate that the inhibitory effects of MCS on EMG are not dependent solely on changes in afferent feedback caused by the muscle twitch produced by the MEP, or on Renshaw cell inhibition. We suggest that some of the inhibitory and excitatory effects of MCS on the motor system are mediated by distinct cortical elements, which may have different susceptibilities to pathophysiological processes in MND.

Adult↗
PubMed1992

Comparison of electric and magnetic coil stimulation in the supraclavicular region.

We compared the compound motor action potentials (CMAPs) evoked in the biceps, triceps, and abductor digiti minimi (ADM) muscles by conventional electrical stimulation at Erb's point (EP), and by magnetic coil stimulation of the supraclavicular region in 11 normal subjects. We found that magnetic coil stimulation was less effective than conventional stimulation in activating motor fibers in the brachial plexus in 45% of the recordings analyzed. CMAP amplitudes greater than those obtained with EP electrical stimulation were seen in 16% of recordings with supraclavicular magnetic stimulation, and in 33% of recordings with cervical magnetic stimulation, indicating that EP electrical stimulation is submaximal in a large proportion of cases.

Action Potentials↗
PubMed1992

Lumbosacral nerve root stimulation comparing electrical with surface magnetic coil techniques.

Stimulation of lumbosacral nerve roots using a monopolar needle electrode was compared with magnetic stimulation using a 7-cm diameter surface coil. Compound muscle action potentials were recorded from the tibialis anterior (TA) and flexor hallucis brevis (FHB) muscles. Although the mean latency of CMAPs did not differ using the two techniques, amplitudes were considerably larger using a needle. Mean amplitudes were 66% (TA) and 64% (FHB) of the direct M response obtained by distal, supramaximal stimulation compared with mean values using maximal magnetic coil stimulation of 36% (TA) and 25% (FHB). Minimum F-wave latencies from FHB were used to estimate the site of nerve root stimulation using both techniques. Although there was a large amount of variability in the data from individual subjects, the results suggested that, on the average, both forms of stimulation act proximal to the intervertebral foramen. We conclude that a needle electrode is a more suitable technique for stimulating lumbosacral nerve roots.

Action Potentials↗
PubMed1992

Familial bilateral carpal tunnel syndrome: report of two families.

Cases of familial carpal tunnel syndrome without other associated conditions are rare. We report two families in which multiple members had bilateral carpal tunnel syndromes. The pattern was consistent with autosomal dominant inheritance. Electrophysiologic studies were performed on nine of the 15 patients, and they demonstrated bilateral pathology of the median nerves at the wrist in all but one patient, without evidence for subclinical, generalized peripheral neuropathy. Quantitative sensory testing was performed in two cases, and it corroborated the absence of peripheral neuropathy. Five of the six patients who underwent carpal tunnel release improved after surgery.

Adolescent↗
PubMed1992

Novel mutations in families with unusual and variable disorders of the skeletal muscle sodium channel.

Mutations in the skeletal muscle sodium channel gene (SCN4A) have been described in paramyotonia congenita (PMC) and hyperkalaemic periodic paralysis (HPP). We have found two mutations in SCN4A which affect regions of the sodium channel not previously associated with a disease phenotype. Furthermore, affected family members display an unusual mixture of clinical features reminiscent of PMC, HPP and of a third disorder, myotonia congenita (MC). The highly variable individual expression of these symptoms, including in some cases apparent non-penetrance, implies the existence of modifying factors. Mutations in SCN4A can produce a broad range of phenotypes in muscle diseases characterized by episodic abnormalities of membrane excitability.

Adult↗
PubMed1992

Motor nerve inexcitability in Guillain-Barré syndrome. The spectrum of distal conduction block and axonal degeneration.

We studied 34 patients with the Guillain-Barré syndrome (GBS) to clarify the clinical significance of inexcitable motor nerves and of low amplitude compound muscle action potentials (CMAPs). The patients were subdivided into two groups. Group 1 included eight patients who had electrically inexcitable motor nerves within 2 wks of the first symptom. (Two patients without extensive conduction studies had only one inexcitable motor nerve.) The outcome in this group at 1 yr varied from complete recovery (five patients) to severe motor sequelae (three patients). Group 2 included 26 patients who had two electrophysiological assessments, and in whom the serial changes in CMAP amplitudes were analysed and correlated to outcome. Fourteen of these 26 sets of serial studies were performed within 1 mth. Twelve of 26 patients in Group 2 showed decrease in the amplitude of CMAPs between serial studies; only six of these had a good outcome at 1 yr. Nine of 26 patients showed increase in CMAP amplitude between serial studies, of these eight had a good clinical outcome. Low-amplitude CMAPs or inexcitable motor nerves in the initial stages of GBS are due to distal pathology of the motor axons, either distal conduction block or axonal degeneration. The nature of these changes cannot be predicted by the results of the initial electrophysiological evaluation, including the presence or absence of active denervation. However, improvement of CMAP amplitude on sequential studies suggests a good outcome at 1 yr. We believe that, in the absence of a biological marker for GBS, individualization of an 'axonal variant' of the syndrome is not warranted at the present time.

Action Potentials↗
PubMed1992

Respiratory failure revealing mitochondrial myopathy in adults.

Two patients, a 70-yr-old black woman and a 56-yr-old black man, presented with respiratory failure unexplained by intrinsic lung disease. Both had been dependent on a respirator for several weeks. No abnormalities of the central or peripheral nervous system or long-standing muscle weakness was noted. The findings from ophthalmologic and cardiac evaluations were normal. The serum creatinine kinase concentration was mildly elevated in case 1, and needle electromyography showed myopathic potentials in case 2. In both instances, muscle biopsy established the diagnosis of mitochondrial myopathy. Biochemical studies of muscle extracts showed partial deficiency of complex 3 in patient 2 and of complex 4 in patient 1. Both patients were weaned from the ventilator after long periods of ventilatory assistance. These observations document a hitherto undescribed presentation of adult-onset mitochondrial myopathy.

Aged↗
PubMed1992

The Lambert-Eaton myasthenic syndrome: a cause of delayed recovery from general anesthesia.

A 70-year-old man required prolonged ventilation after surgery to remove a rectal neoplasm. The cause of the slow recovery from the effects of neuromuscular blocking agents used during his anesthetic was the Lambert-Eaton myasthenic syndrome (LEMS). Before surgery, he had no neuromuscular symptoms, even in retrospect. LEMS should be considered in the diagnosis of prolonged recovery from neuromuscular blockade, even in previously asymptomatic patients.

Adenocarcinoma↗
PubMed1991

Hemispheric threshold differences for motor evoked potentials produced by magnetic coil stimulation.

A brief monophasic pulse through an electromagnetic coil preferentially activates motor pathways of each hemisphere, depending on the direction of coil current flow. Using the preferred direction for each hemisphere, the minimum stimulus intensity (threshold) that evoked compound muscle action potentials in the contralateral abductor digiti minimi (ADM) muscle was significantly less for the left hemisphere than the right. Threshold for biceps on each side was significantly higher than ADM, but there was no side-to-side difference. Assessing handedness using a standard handedness index, those who had less tendency to use the right hand for everyday tasks had greater differences between hemispheres for ADM thresholds. The lower threshold of the left-hemisphere projection to hand muscles is probably related to the asymmetry of corticomotoneuronal monosynaptic connections; a greater number project to the motor neuron pool of the right- than left-hand muscles.

Action Potentials↗