CSF amino acids and plasma--CSF amino acid ratios in adults.
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Biomedical subjects
Publications and source records attributed to S Hansen.
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Certain differences between phage T3 on the one hand and T3sam- and T7 on the other hand indicate that the T3-coded SAMase function is responsible (i) for the development of the pseudolysogenic state by preventing T3 DNA methylation, and (ii) for the partial protection of the phage DNA against restriction by the P system.
Single motor unit potentials recorded from surface electrodes over the extensor digitorum brevis muscle and evoked by stimulation of the anterior tibial nerve at the ankle were obtained by a computer subtraction method. Their latencies, durations, amplitudes, and areas were measured in control subjects and patients with Duchenne, limb-girdle, facioscapulohumeral, and myotonic muscular dystrophy. Lateral popliteal motor nerve conduction velocities were also recorded. In the muscular dystrophies there was a significant increase in both the latencies and durations of motor unit potentials, the latter in notable contrast with the findings of conventional needle electromyography. Fastest motor conduction velocities were significantly reduced in the limb-girdle, facioscapulohumeral, and myotonic muscular dystrophy patients, while the shortest distal motor latencies were significantly prolonged in these patients and those with Duchenne muscular dystrophy. The results support the presence of a definitive neurogenic influence in the muscular dystrophies.
We have described two siblings who excrete massive amounts (up to 3.89 mmol/24 hr) of N-formiminoglutamic acid (FIGLU) in their urine. This unusual compound was isolated from urine, purified, and firmly identified as FIGLU by combined gas chromatography-mass spectrometry. The patients presumably have a deficiency in activity of the hepatic enzyme, glutamate formiminotransferase, which carries out the fourth sequential step in the main pathway of histidine degradation. Unlike children reported previously with this disorder, our patients had normal serum folate levels, had no hematologic abnormalities, and were not mentally retarded. Very small amounts of FIGLU were present in the plasma of one of the patients, but FIGLU was not detectable in the cerebrospinal fluid of either patient. Administration of pharmacologic doses of folic or folinic acid produced a decrease in excretion of FIGLU in urine. Histidine loading caused a small and comparable urinary excretion of FIGLU in the children's parents and in control adult subjects.
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An on-line computer method is described for the isolation of single motor unit potentials, evoked by stimulation of the anterior tibial nerve at the ankle, from the compound action potential recorded from surface electrodes over the extensor digitorum brevis muscle. The latencies, durations, amplitudes, and areas of the potentials were measured in a group of control subjects and patients with myasthenia gravis. In myasthenic patients there is a significant reduction in the durations of motor unit potentials and a significant increase in latencies while their amplitudes and areas remain unchanged. The results are consistent with the presence of a `terminal neuropathy' in myasthenia gravis.
The results of the application of a computerized method for the estimation of motor unit numbers in the human extensor digitorum brevis are presented. In patients with Duchenne and limb-girdle and facioscapulohumeral muscular dystrophies, motor unit numbers are within the normal range, but are significantly reduced in myotonic muscular dystrophy.
A new method, incorporating on-line computer analysis, is described for the estimation of the numbers of motor units in human muscle. The results obtained in the extensor digitorum brevis muscle in normal subjects and patients with myasthenia gravis are presented. These indicate that the numbers of motor units in that muscle in patients with myasthenia gravis are within the normal range, in contrast with the reduction in numbers reported by other workers using a different technique. Evidence is presented to suggest that the discrepancy in these results is due to increased sensitivity and discrimination of the computerized method. Several hypotheses on the aetiology of a number of neuromuscular diseases, based on the results of the other method, may require reevaluation.
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