[Clinical neurophysiological evaluation of the motor unit].
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Electromyography↗
Biomedical subjects
E Stålberg
Publications and source records attributed to E Stålberg.
An 8-year longitudinal study of muscle strength, muscle fiber size, and dynamic electromyogram in individuals with late polio.
Twenty-one subjects with polio 24 to 51 years prior to the first examination were studied on three occasions, each 4 years apart with measurements of muscle strength and endurance for knee extension, macro EMG, and muscle biopsy from vastus lateralis. On average the muscle strength decreased during the 8-year follow-up by 9-15%. Endurance decreased during the observation period. The muscle fiber area was markedly increased in most subjects. There was a decrease in the capillarization during the follow-up. Macro EMG was increased in all subjects (range 3-42 times control) and increased in 20 legs during the 8-year follow-up, but showed a decrease in 8 of 9 legs with an approximative breakpoint when macro MUPs were around 20 times the normal size. Thus, evidence of on-going denervation/reinnervation as well as of failing capacity to maintain large motor units was demonstrated. SFEMG showed a moderate degree of disturbed neuromuscular transmission.
Adult↗
Association of muscle strength and electrophysiological measures of reinnervation in diabetic neuropathy.
Motor function was assessed in 34 non-insulin-dependent and 19 insulin-dependent diabetic patients with macroelectromyography and isokinetic dynamometry. Fiber density (FD) and the amplitude of the macro motor unit potential (macro MUP) of the anterior tibial and lateral vastus muscles were obtained and maximal isokinetic strength of the ankle and knee extensors were determined. All patients underwent standardized clinical examination including a neurological disability score (NDS), quantitative sensory examination, and conventional motor nerve conduction studies. The amplitude of the macro MUP and FD of the anterior tibial muscle were increased in neuropathic patients without weakness (P < 0.05) and further increased in neuropathic patients with weakness (P < 0.05). The NDS was related to the FD and the amplitude of the macro MUP for the anterior tibial and lateral vastus muscle [r=0.55-0.75 (P < 0.005)]. Muscle strength of ankle and knee extensors correlated with the FD [r=-0.69 (P < 0.0001) and r=-0.58 (P< 0.001), respectively] and with the amplitude of the macro MUP of the two muscles [r=-0.63 (P < 0.0001) and r=-0.37 (P < 0.05), respectively]. Our findings support the hypothesis that loss of muscle strength in diabetic patients is due to incomplete reinnervation following axonal loss.
Adult↗
Spinal cord evoked potentials and edema in the pathophysiology of rat spinal cord injury. Involvement of nitric oxide.
The possibility that nitric oxide is somehow involved in the early bioelectrical disturbances following spinal cord injury in relation to the later pathophysiology of the spinal cord was examined in a rat model of spinal cord trauma. A focal trauma to the rat spinal cord was produced by an incision of the right dorsal horn of the T 10-11 segments under urethane anaesthesia. The spinal cord evoked potentials (SCEP) were recorded using epidural electrodes placed over the T9 and T12 segments of the cord following supramaximal stimulation of the right tibial and sural nerves in the hind leg. Trauma to the spinal cord significantly attenuated the SCEP amplitude (about 60%) immediately after injury which persisted up to 1 h. However, a significant increase in SCEP latency was seen at the end of 5 h after trauma. These spinal cord segments exhibited profound upregulation of neuronal nitric oxide synthase (NOS) immunoreactivity, and the development of edema and cell injury. Pretreatment with a serotonin synthesis inhibitor drug p-chlorophenylalanine (p-CPA) or an anxiolytic drug diazepam significantly attenuated the decrease in SCEP amplitude, upregulation of NOS, edema and cell injury. On the other hand, no significant reduction in SCEP amplitude, NOS immunolabelling, edema or cell changes were seen after injury in rats pretreated with L-NAME. These observations suggest that nitric oxide is somehow involved in the early disturbances of SCEP and contribute to the later pathophysiology of spinal cord injury.
Animals↗
House-Brackmann and Yanagihara grading scores in relation to electroneurographic results in the time course of Bell's palsy.
The results of House Brackmann and Yanagihara grading were compared with electroneurographic (ENoG) data in 30 consecutive patients with Bell's palsy. The examinations were made on mean days 11, 36 and 99. Twenty-four patients had a favourable outcome (Yanagihara > or = 36 at three months). Based on our observations, 23 (96%) of these could have been predicted by ENoG, 18 (75%) by Yanagihara grading and 6 (25%) by House Brackmann grading. Initially, the relative House Brackmann scores showed a slightly milder palsy than the Yanagihara scores, but in the follow-up period the gradings were almost identical. The mild palsies, defined on the initial ENoG results, initially demonstrated relatively less nerve dysfunction on ENoG than the clinical grading; by the first follow-up, the ENoG and clinical grading had both returned to normal. The intermediate palsies had almost the same initial relative clinical and ENoG values, but at the first follow-up (mean day 36), the facial function had returned to normal despite abnormally reduced, but improved, ENoG values. In the severely affected patients, the follow-up studies showed an improved clinical function but ENoG values still demonstrated a high degree of degeneration (slightly improved at second follow-up). In this study, patients with a favourable outcome were best predicted with ENoG. Clinical identification of these patients was more accurate with Yanagihara than with House Brackmann. Furthermore, in all three groups a clinical improvement, due to the release of neurapraxia, was noted at the first follow-up. The slow ENoG improvement noted at follow-up was probably due to nerve regeneration by collateral sprouting. Based on the time course of our ENoG findings, it appears that patients with a high degree of degeneration at both the initial examination and first follow-up have a poorer prognosis.
Adult↗
The study of normal and abnormal neuromuscular transmission with single fibre electromyography.
The use of single fibre electromyography (SFEMG) in the study of neuromuscular transmission across individual motor endplates in situ is reviewed. The neuromuscular jitter can be studied both during voluntary contraction and electrical activation of the muscle fibre. The differences, pitfalls and advantages of these methods are discussed. Findings in myasthenia gravis and other disorders of the neuromuscular transmission are examined.
Electric Stimulation↗
F-wave latency, the most sensitive nerve conduction parameter in patients with diabetes mellitus.
In this study we examined the diagnostic sensitivity of minimal F-wave latency, F-wave persistence, motor nerve conduction velocity (MCV), and amplitude of the compound motor action potential (CMAP) of the median, ulnar, tibial, and peroneal nerves, and of sensory conduction velocity (SCV) and sensory nerve action potential (SNAP) amplitude of the sural nerve in 82 diabetic patients. For the median, ulnar, and tibial nerves the Z scores of the minimal F-wave latency were significantly larger than those of the MCV, and for all four motor nerves the Z scores of the minimal F-wave latency were significantly larger than those of the amplitude of the CMAP. The Z scores of the peroneal minimal F-wave latency exceeded those of peroneal MCV, sural SCV, and sural SNAP. F-wave persistence did not differ significantly from the reference values. In conclusion, minimal F-wave latency is the most sensitive measure for detection of nerve pathology and should be considered in electrophysiological studies of diabetic patients.
Action Potentials↗
Postpolio muscular dysfunction: relationships between muscle energy metabolism, subjective symptoms, magnetic resonance imaging, electromyography, and muscle strength.
Eleven patients with previous polio were studied. The concentration of energy-related metabolites and energy charge was measured from the vastus lateralis muscle, as was isometric muscle strength of knee extension. Cross-sectional area of the quadriceps femoris muscle was calculated from magnetic resonance imaging. Reinnervation was studied using macroelectromyography. Muscle weakness, pain, and newly acquired muscle weakness in the legs was estimated by the patients. The findings in the legs in which the patients experienced new loss of muscle function were compared with the stable legs. There were no significant differences between these groups in any of the objectively measured variables. Only hip pain correlated with new loss of muscle function. Creatine phosphate was decreased in 5 patients. The symptoms and subjective muscle strength did not correlate with any of the objective measurements. There were no significant relationships between energy-related metabolites and postpolio symptoms.
Adult↗
Benzodiazepine receptors influence spinal cord evoked potentials and edema following trauma to the rat spinal cord.
The possibility that diazepam will influence spinal cord evoked potentials (SCEP), edema formation and cell changes following spinal cord injury (SCI) was examined in a rat model. The SCI was produced in equithesin anaesthetised animals by making a longitudinal incision (about 2 mm deep and 5 mm long) in the right dorsal horn of the T10-11 segments. The SCEP were recorded from the epidural space of the T9 segment after stimulation of the right tibial and sural nerves. The SCEP consisted of a small positive peak followed by a broad and high negative peak. Infliction of trauma to the rats resulted in an immediate and pronounced decrease of the maximal negative peak (MNP) amplitude. The spinal cord edema and cell changes were markedly pronounced 5 h after injury. Pretreatment with diazepam attenuated the early SCEP changes induced by the trauma and reduced the later development of edema and cell injury. These results suggest that benzodiazepine receptors are involved in trauma induced alterations in SCEP changes, edema formation and cell injury, not reported earlier.
Animals↗
The role of electromyography in neurology.
A review is given of the role of electromyography (EMG) for diagnosis, pathophysiological description and monitoring of patients with disorders of the peripheral nervous system. Various EMG methods are presented and their principal differences are discussed. The usefulness of these methods varies depending on the pathology to be studied. With modern quantitative methods for analysis, EMG has become more sensitive and accurate and is therefore an important part in the evaluation of the neurologic patient. EMG results are usually combined with findings from other neurophysiological investigations (neurography, evoked potentials), histochemistry, biochemistry and most importantly with the clinical signs to give as complete a picture of the condition as possible. The usefulness of EMG depends on a number of factors other than the quality of the investigation as such. These aspects are discussed briefly.
Electromyography↗
Serial gadolinium-enhanced magnetic resonance imaging and assessment of facial nerve function in Bell's palsy.
Eleven patients with mild or moderate acute idiopathic peripheral facial palsy, so-called Bell's palsy, were serially examined by gadolinium-DTPA-enhanced MRI on mean days 11, 40, and 97 (third examination, n = 10) after the onset of palsy. Results of the clinical and neurophysiologic assessment of facial nerve function were compared with the gadolinium-enhanced MRI findings. Eight of the 11 patients demonstrated contrast enhancement of the facial nerve at the initial examination, but in 7 of them, the enhancement had disappeared by the time of the serial follow-up gadolinium-enhanced MRI scans. The disappearance of facial nerve enhancement was found to be related to clinical and neurophysiologic improvements in facial nerve function during recovery from Bell's palsy. The three patients whose scans were negative at the initial gadolinium-enhanced MRI examination had the same clinical severity of palsy, but initially they had milder neurophysiologic involvement than those who demonstrated enhancement; these three patients did not exhibit enhancement at serial follow-up scans. These findings indicate that the presence of enhancement at the initial MRI scan is not necessarily indicative of a poor prognosis for recovery.
Acute Disease↗
A special kind of anterior horn cell involvement in juvenile myoclonic epilepsy demonstrated by macro electromyography.
Juvenile myoclonic epilepsy (JME) is not an uncommon seizure disorder, occurring in 5-10% of epileptic patients. A subclinical anterior horn cell involvement has been suggested in some JME patients by concentric needle electromyography (EMG) and turn/amplitude analysis. In this study, 22 JME patients and 17 normal control subjects have been studied with macro EMG, which is a sensitive method to assess the size of motor units. Most JME patients (19 of 22) had a pathologically increased number of individual large macro motor unit action potentials (MUAPs) compared to control subjects. For both biceps brachii and tibialis anterior muscles, means of median macro MUAP amplitudes were significantly greater than those of normal controls, whereas the fiber density values were only slightly increased. This suggested another kind of anterior horn cell involvement in JME than seen in motor neuron diseases.
Action Potentials↗
[Children with poliomyelitis in Sweden 1996].
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Africa↗
Muscle-nerve involvement in autosomal dominant progressive external ophthalmoplegia with hypogonadism.
Sixteen members of a family with a history of autosomal dominant progressive external ophthalmoplegia (adPEO) with hypogonadism were examined. The muscular involvement commenced cranially and descended in relation to increasing disease duration. The neuromuscular signs were PEO, dysarthria, dysphonia, limb muscle weakness with wasting, absence of Achilles tendon reflexes, and distal vibration sensory loss. The electromyogram (EMG) was myopathic in facial and proximal limb muscles. Neurogenic involvement was suspected in a few tibial anterior muscles. Neurography showed signs of axonal neuropathy correlated to clinical signs. F-responses were reduced in number or absent in peroneal nerves, and did not correlate to clinical signs or disease duration. Muscle biopsies in advanced cases had structural abnormalities of mitochondria, ragged-red fibers, and focal cytochrome c oxidase deficiency. A combination of muscle-nerve involvement with PEO, Achilles tendon areflexia, distal vibration sensory impairment, myopathic EMG, and abnormally low sural nerve responses seems to be typical of this type of mitochondrial disorder.
Adolescent↗
Anticipation of autosomal dominant progressive external ophthalmoplegia with hypogonadism.
A large Swedish family with members affected by progressive external ophthalmoplegia with hypogonadism were followed-up and reviewed. Hypogonadism included delayed sexual maturation, primary amenorrhea, early menopause, and testicular atrophy. Cataracts, cerebellar ataxia, neuropathy, hypoacusia, pes cavus, tremor, parkinsonism, depression, and mental retardation were other features observed in this family. Muscle biopsy samples of advanced cases showed ragged-red fibers, focal cytochrome c oxidase deficiency, and multiple mtDNA deletions by Southern blot analysis. An autosomal dominant mode of inheritance was evident with anticipation in successive generations. Linkage analysis excluded the chromosome 10q23.3-q24.3 region reported as being linked to the disease in a Finnish family with autosomal dominant progressive external ophthalmoplegia. We report for the first time clinical evidence for anticipation in a family with autosomal dominant progressive external ophthalmoplegia. We hypothesize that the nuclear gene causing this enigmatic disorder may be directly influenced by an expansion of an unstable DNA sequence and that the resulting phenotype is caused by a concerted action with multiple deletions of mtDNA.
Adult↗
Generator sites for spontaneous activity in neuromyotonia. An EMG study.
A 16-year-old female patient with symptoms and signs compatible with neuromyotonia was studied with various neurophysiological tests and with muscle biopsy. Nerve conduction studies revealed signs of axonal motor neuropathy. EMG showed denervation in distal muscles, and moderate neurogenic changes in other muscles. Abundant spontaneous motor unit activity was recorded in all muscles. This activity did not disappear upon proximal nerve blockade with local anaesthetics. Based on the shape of spontaneous discharges and their behaviour on nerve stimulation and during voluntary effort, the site of generation was suggested. This varied for different discharges, from proximally in the nerve, to various sites along the intramuscular nerve tree. In some axons there were signs of conduction block proximal to the generation site for the spontaneous discharges. Different axons showed various degrees of abnormality; local hyperexcitability triggering new impulses only after the passage of a preceding impulse, increased hyperexcitability generating spontaneous activity, total impulse blocking, and finally axonal degeneration. Treatment with dihydantoin reduced the spontaneous activity with concomitant clinical improvement.
Adolescent↗
Significance of A-waves recorded in routine motor nerve conduction studies.
The occurrence of A-waves during routine F-wave studies was investigated in 556 consecutive patients referred to the Department of Clinical Neurophysiology at the University Hospital in Uppsala for various neuromuscular disorders. Altogether, 2367 nerves in the upper and lower extremities were studied. An A-wave, with a nearly constant latency and a uniform shape on consecutive stimulations, could be recorded in 184 nerves (7.8%) out of 124 patients (22.3%). More than 50% of patients with A-waves had various types of polyneuropathies. Of all patients with polyneuropathy, 65% had at least one nerve with A-waves. A-waves occurred somewhat less frequently in patients with radiculopathies. In other proximal local nerve lesions they were found less often and only exceptionally in patients with distal nerve lesions. A-waves were present in 6 out of 10 patients with motor neurone diseases. There was no correlation between the number of A-waves found in one nerve or the number of nerves in a given patient with A-waves and the aetiology or severity of the underlying disease. A-waves were found in 11 patients referred for various neurological symptoms in whom other neurophysiological findings were normal. This might be interpreted as an early sign of underlying disease because in 100 healthy controls no A-waves could be elicited, with the exception of 3 subjects who had A-waves in the abductor hallucis muscle when the tibial nerve was stimulated. We conclude that the appearance of A-waves should be considered a sign of either a local nerve lesion or a generalised neuropathy in all other nerves except for the tibial nerve.
Electromyography↗
Quantitative motor unit potential analysis.
A review of quantitative methods for electromyography is given. Background information about motor unit anatomy, physiology, and pathology is provided to explain some of the presented electrophysiological phenomena. Different aspects of quantitation, such as motor unit action potential parameters, automatic analysis methods, reference values, and findings in abnormal conditions, are discussed.
Axons↗