Electrophysiological and clinical correlations in the Lambert-Eaton myasthenic syndrome.
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Biomedical subjects
Publications and source records attributed to G Claussen.
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We have developed a repetitive nerve stimulation (RNS) technique for the peroneal nerve. Normal limits for the decremental responses for the anterior tibialis and extensor digitorum brevis muscles are 6-21% at the low rate of stimulation and 44-70% at the high rate of stimulation. These values exceed the normal limits for other commonly tested muscles. This may be due to the lower safety factor for neuromuscular transmission for the anterior tibialis and extensor digitorum brevis muscles. We present 4 cases in which the peroneal nerve RNS test was crucial for the diagnosis of the limb-girdle form of MG or LEMS. Thus, we conclude that, in a small number of patients with neuromuscular transmission disorders, the peroneal nerve RNS test is needed for confirmation of disease.
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Myasthenia gravis can present with rapid respiratory failure as the first manifestation of disease. In the Lambert-Eaton myasthenic syndrome (LEMS), such a manifestation has rarely been reported. We are reporting a patient who developed respiratory failure as the first manifestation of LEMS without associated carcinoma.
13 new and 19 explanted hydrocephalus shunt valves were tested under perfusion for two weeks, using a modified ASTM test. Some manufacturers seem to have problems with quality control. Our results show large deviations in pressure-flow-characteristics in different valves, in some cases leading to an extremely high pre-shunt pressure. Test results almost corresponding to manufacturer's specifications were found in diaphragm valves and in some ball and spring valves. Results from slit valves usually varied widely, some being good, but others bad (much too high a pressure) or dangerous with even possibly fatal characteristics. Whilst laboratory performance may not correlate with clinical results, due to different impact of cerebrospinal fluid (CSF) pressure and flow on hydrocephalus shunting systems, manufacturers should provide devices that meet their supposed specifications. We developed an apparatus allowing simulation of different CSF production rates, CSF outflow resistances, CSF pressure waves caused by arterial and venous pulsation, breathing and coughing, and hydrostatic differential pressure caused by body position changes. This model enables testing of shunt valves under almost physiological conditions, thus closing the gap between conventional bench test results and clinical performance of hydrocephalus shunt valves.
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