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Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report
BACKGROUND: Lambert-Eaton myasthenic syndrome (LEMS) is a type of paraneoplastic syndrome that may initially manifest itself with proximal weakness and gait abnormalities. Approximately up to 50% of LEMS patients have a primary autonomic dysfunction. CASE PRESENTATION: We present here a case of a 75...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8933941/ https://www.ncbi.nlm.nih.gov/pubmed/35305594 http://dx.doi.org/10.1186/s12883-022-02625-1 |
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author | Zhang, Jinghong Huang, Xusheng Shi, Qiang |
author_facet | Zhang, Jinghong Huang, Xusheng Shi, Qiang |
author_sort | Zhang, Jinghong |
collection | PubMed |
description | BACKGROUND: Lambert-Eaton myasthenic syndrome (LEMS) is a type of paraneoplastic syndrome that may initially manifest itself with proximal weakness and gait abnormalities. Approximately up to 50% of LEMS patients have a primary autonomic dysfunction. CASE PRESENTATION: We present here a case of a 75-year-old male with symmetric proximal muscle weakness, dry mouth and constipation. The cutaneous response to scratch and upright tilt-table testing were positive. A repetitive nerve stimulation test showed that there was a decremental response of compound muscle action potential (CMAP) amplitude at 3 Hz while an incremental response at 20 Hz. The presence of antibodies against voltage-gated calcium channels (VGCC) confirmed the diagnosis. Because of the prominent symptom of autonomic disorder, the patient further underwent the test of skin sympathetic response (SSR). Lower amplitude and longer response duration were found in palms, while it evoked no response in soles. CONCLUSIONS: In this case, we present the detailed results of SSR test on a patient suffering LEMS with autonomic disorder. Since autonomic dysfunction has a significant impact on clinical management and SSR test is an effective detection method, we recommend that SSR test be performed on patients with LEMS regularly. |
format | Online Article Text |
id | pubmed-8933941 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-89339412022-03-23 Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report Zhang, Jinghong Huang, Xusheng Shi, Qiang BMC Neurol Case Report BACKGROUND: Lambert-Eaton myasthenic syndrome (LEMS) is a type of paraneoplastic syndrome that may initially manifest itself with proximal weakness and gait abnormalities. Approximately up to 50% of LEMS patients have a primary autonomic dysfunction. CASE PRESENTATION: We present here a case of a 75-year-old male with symmetric proximal muscle weakness, dry mouth and constipation. The cutaneous response to scratch and upright tilt-table testing were positive. A repetitive nerve stimulation test showed that there was a decremental response of compound muscle action potential (CMAP) amplitude at 3 Hz while an incremental response at 20 Hz. The presence of antibodies against voltage-gated calcium channels (VGCC) confirmed the diagnosis. Because of the prominent symptom of autonomic disorder, the patient further underwent the test of skin sympathetic response (SSR). Lower amplitude and longer response duration were found in palms, while it evoked no response in soles. CONCLUSIONS: In this case, we present the detailed results of SSR test on a patient suffering LEMS with autonomic disorder. Since autonomic dysfunction has a significant impact on clinical management and SSR test is an effective detection method, we recommend that SSR test be performed on patients with LEMS regularly. BioMed Central 2022-03-19 /pmc/articles/PMC8933941/ /pubmed/35305594 http://dx.doi.org/10.1186/s12883-022-02625-1 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Zhang, Jinghong Huang, Xusheng Shi, Qiang Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report |
title | Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report |
title_full | Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report |
title_fullStr | Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report |
title_full_unstemmed | Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report |
title_short | Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report |
title_sort | autonomic dysfunction detected by skin sympathetic response in lambert-eaton myasthenic syndrome: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8933941/ https://www.ncbi.nlm.nih.gov/pubmed/35305594 http://dx.doi.org/10.1186/s12883-022-02625-1 |
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