The electrodiagnosis of myasthenia gravis.
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Biomedical subjects
Publications and source records attributed to D B Sanders.
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The electromyographic (EMG) interference pattern (IP) was simulated by adding together motor unit action potentials (MUAPs) of different sizes that had been recorded by a concentric needle EMG electrode. The number of turns (NT) of the simulated IP increased with the number of MUAP discharges. The mean amplitude (MA) difference between successive turns in the IP increased when large amplitude MUAPs were added. Our analysis demonstrates that the MA of the IP is determined mainly by the amplitude of large MUAPs in the signal and that large amplitude spikes are more likely to be generated by single large amplitude MUAPs than by summation of several small amplitude MUAPs.
We have developed three new features of the electromyographic interference pattern (IP), based on the turns and amplitude of the signal, to quantitate some of the features of the IP that are usually assessed subjectively by an electromyographer. The activity measures the fullness of the IP. The upper centile amplitude (UCA) defines the upper limit of the maximum peak-to-peak amplitude of the motor unit action potentials (MUAPs) contained in the IP. The number of small segments (NSS) measures the complexity of the IP, which is a reflection of the polyphasicity of the component MUAPs. The activity and the logarithm of the UCA correlate strongly with the force of muscle contraction at which the IP is measured. The NSS initially increases with the force of contraction and becomes relatively constant at higher force levels. The normal values of these features and the interpretation of their relationships are described in companion papers.
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In four patients who received periocular injections of botulinum toxin for blepharospasm, abnormal neuromuscular transmission was demonstrated by single-fiber EMG in arm muscles. The time course with which the abnormalities developed and cleared, as well as the inverse relationship between the neuromuscular jitter and the firing rate in the abnormal muscles, indicated that the toxin caused the abnormalities in arm muscles. No weakness was detected clinically in muscles distant from the face, but the abnormal neuromuscular transmission indicates that the toxin spread remotely from the site of injection.
A 10-year-old girl was treated for an acute asthmatic attack with ventilation and a high-dose steroids regimen. An areflexic paralysis of the four limbs was noted when artificial ventilatory support was withdrawn. Electromyography and a muscle biopsy revealed marked myopathic features. We discuss the unusual presentation of an acute steroid-induced myopathy in an asthmatic patient.
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Symptoms of McArdle's disease (muscle phosphorylase deficiency) commonly begin in childhood or adolescence. Late onset of the disease is rare. We describe a 76-year-old man whose symptoms began at age 74 years with sudden onset of proximal muscle weakness and fatigability. Electromyography disclosed substantial spontaneous activity and myopathic features as seen in inflammatory muscle disease. The diagnosis of McArdle's disease was made by histochemical studies of muscle, an abnormal ischemic lactate test, and absence of myophosphorylase activity.
Patients with myasthenia gravis (MG) have increased tolerance to the neuromuscular blocking properties of suxamethonium (SCh) and decamethonium (C10) and exhibit a reversal of the C10-induced block by neostigmine. The effects of these drugs were compared in forelimb flexor digitorum longus muscle from normal rats and from rats with experimental autoimmune myasthenia gravis (EAMG) to investigate the similarity of EAMG to MG. The depolarization induced by 1, 5, 10 and 25 microM SCh or C10 at the motor end-plates was significantly higher in normal than in EAMG muscle. However, both normal and EAMG end-plates responded in a similar qualitative manner to each drug. The depolarization produced by SCh was typically maintained until the drug was washed from the bath. The depolarization produced by C10 tended to decrease after reaching its peak despite continued application of the drug. With both drugs, miniature end-plate potential (MEPP) amplitude reduction is maintained until a saline wash. Neostigmine interaction with SCh and C10 in normal and EAMG muscle was compared by measuring isometric twitch tension in vitro. Neostigmine potentiated the neuromuscular block produced by either SCh or C10 in both normal and EAMG muscle. Thus muscle from rats with EAMG shares with MG an increased tolerance to SCh and C10 when compared to normal muscle but does not exhibit the qualitatively different interaction of C10 affected muscle with neostigmine that is found in MG patients. This and other studies comparing EAMG and MG indicate that EAMG is an appropriate model of MG but differences such as we have noted should be considered when extrapolating data from EAMG to the human disease.
A simple biological signal generator capable of reproducing complex biopotential waveforms is described. It is constructed by a combination of digital and analog circuit components and can be used under different experimental conditions, such as in calibration of biomedical instrumentation systems, or simply as a function generator providing voltage outputs of various waveforms. The biopotential waveform to be generated is sampled at a high frequency and the samples are stored sequentially in a programmable read only memory (PROM). The samples are then fed in the same sequence to a digital-to-analog (D/A) converter and the resulting output is amplified and a DC offset is added. External controls are provided to adjust the DC offset, amplitude and repetition rate of the signal generated. The reproduced voltage signals are stable and superior in quality to those produced by conventional biological signal generators.
The pattern of stapedial reflex fatigue in response to pulsed acoustic stimulation was measured and compared to results of repetitive nerve stimulation and single-fiber electromyography (EMG) in 89 patients with myasthenia gravis. Studies were also made on 22 patients with other neuromuscular disorders and 40 control subjects with no evidence of neuromuscular impairment. Stapedial reflex fatigue exceeded normal control values in 84% of the patients with myasthenia gravis. Repetitive stimulation and single-fiber EMG measurements were abnormal in 56% and 91% of this same population, respectively. Stapedial reflex abnormalities were most prevalent in patients with mild forms of myasthenia (predominantly ocular or oropharyngeal weakness). Of 22 nonmyasthenic patients with neuromuscular disease tested, 6 had abnormal stapedial reflex fatigue according to our normal values, indicating that this form of testing also detects other diseases of the motor unit. The measurement of stapedial reflex fatigue is painless, is easy to perform, and requires minimal patient cooperation. Due to the relatively high occurrence of abnormal stapedial reflex fatigue in patients with myasthenia gravis, this procedure appears to have considerable potential value in screening and monitoring patients for the presence of defects in neuromuscular transmission.
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We have found a wide range of mean MEPP amplitude in intercostal muscle biopsies from 43 patients with MG, including several values in the normal range. There was no correlation between MEPP amplitude and the severity of clinical disease as assessed by manual muscle testing or by single-fiber EMG measurements of jitter in arm muscles. Through most of these patients were in a state of clinical remission or marked improvement after treatment with prednisone, we could not attribute the difference between our results and those of others to this factor alone. The application of morphine, meperidine and aminoglycoside antibiotics to intercostal muscle in vitro confirms effects previously demonstrated in rat muscle: (1) At equal therapeutic concentrations, meperidine has greater neuromuscular blocking effects than does morphine, but neither has significant effects at concentrations achieved in the serum clinically. (2) Tobramycin, netilmicin and neomycin have varying severity and sites of action, but their effects are the same in human myasthenic muscle as in normal rat muscle. Bath application of serum from myasthenic patients produces an acute, reversible worsening of neuromuscular blockade in myasthenic muscle. Electrophysiologic measurements in intercostal biopsies from patients with MG can provide information about the basic abnormality of neuromuscular transmission in this disease and can confirm the relevance of studies made in animal muscle.
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