Lambert-Eaton syndrome (Q41419): Difference between revisions
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A síndrome miastênica de Lambert-Eaton, 20 vezes mais rara que a miastenia gravis positiva para receptores de acetilcolina, com prevalência de 3,42 por milhão, é uma doença imunomediada da junção neuromuscular. Clinicamente, a doença é caracterizada por fraqueza proximal das pernas. Na maioria dos pacientes, a fraqueza se estende a outros músculos, incluindo os músculos oculobulbares. Os sintomas autonômicos (boca seca, disfunção erétil, constipação) são frequentes. Os reflexos tendinosos estão reduzidos. A estimulação nervosa repetitiva mostra baixos potenciais de ação compostos do músculo, decréscimo > 10% em baixa frequência e incremento > 100% após a contração voluntária máxima em alta frequência. | |||
| description / en | description / en | ||
Lambert-Eaton myasthenic syndrome, 20 times as rare as acetylcholine receptor positive myasthenia gravis with a prevalence of 3.42 per million, is an immune-mediated disease of the neuromuscular junction. Clinically the disease is characterised by proximal weakness of the legs. In most patients, the weakness extends to other muscles including the oculobulbar ones. Autonomic symptoms (dry mouth, erectile dysfunction, constipation) are frequent. Tendon reflexes are reduced. Repetitive nerve stimulation shows low compound muscle action potentials, decrement > 10% at low frequency and increment > 100% after maximum voluntary contraction at high frequency. | |||
Revision as of 07:35, 13 August 2026
Lambert-Eaton myasthenic syndrome, 20 times as rare as acetylcholine receptor positive myasthenia gravis with a prevalence of 3.42 per million, is an immune-mediated disease of the neuromuscular junction. Clinically the disease is characterised by proximal weakness of the legs. In most patients, the weakness extends to other muscles including the oculobulbar ones. Autonomic symptoms (dry mouth, erectile dysfunction, constipation) are frequent. Tendon reflexes are reduced. Repetitive nerve stimulation shows low compound muscle action potentials, decrement > 10% at low frequency and increment > 100% after maximum voluntary contraction at high frequency.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 8C62 |
||
| English | Lambert-Eaton syndrome |
Lambert-Eaton myasthenic syndrome, 20 times as rare as acetylcholine receptor positive myasthenia gravis with a prevalence of 3.42 per million, is an immune-mediated disease of the neuromuscular junction. Clinically the disease is characterised by proximal weakness of the legs. In most patients, the weakness extends to other muscles including the oculobulbar ones. Autonomic symptoms (dry mouth, erectile dysfunction, constipation) are frequent. Tendon reflexes are reduced. Repetitive nerve stimulation shows low compound muscle action potentials, decrement > 10% at low frequency and increment > 100% after maximum voluntary contraction at high frequency. |
