Lambert-Eaton myasthenic syndrome.
| Autor: | Lipka AF; Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands; Department of Neurology, Groene Hart Hospital, Gouda, The Netherlands. Electronic address: a.f.lipka@lumc.nl., Verschuuren JJGM; Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands. |
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| Jazyk: | angličtina |
| Zdroj: | Handbook of clinical neurology [Handb Clin Neurol] 2024; Vol. 200, pp. 307-325. |
| DOI: | 10.1016/B978-0-12-823912-4.00012-8 |
| Abstrakt: | Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disease characterized by proximal muscle weakness, loss of tendon reflexes, and autonomic dysfunction. Muscle weakness usually starts in the upper legs and can progress to oculobulbar and in severe cases respiratory muscles. P/Q-type voltage-gated calcium channels (VGCCs) localized in the presynaptic motor nerve terminal and in the autonomic nervous system are targeted by antibodies in LEMS patients. These antibodies can be detected in about 90% of patients, and the presence of decrement and increment upon repetitive nerve stimulation is also a highly sensitive diagnostic test. Rapid diagnosis is important because of the association with SCLC in 50%-60% of patients, which stresses the need for vigorous tumor screening after diagnosis. Clinical parameters can predict tumor probability and guide frequency of tumor screening. Treatment of the tumor as well as symptomatic treatment and immunosuppression can effectively control symptoms in the majority of patients. (Copyright © 2024 Elsevier B.V. All rights are reserved, including those for text and data mining, AI training, and similar technologies.) |
| Databáze: | MEDLINE |
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