Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Zhang, Jinghong, Huang, Xusheng, Shi, Qiang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
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
_version_ 1784671766064398336
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
work_keys_str_mv AT zhangjinghong autonomicdysfunctiondetectedbyskinsympatheticresponseinlamberteatonmyasthenicsyndromeacasereport
AT huangxusheng autonomicdysfunctiondetectedbyskinsympatheticresponseinlamberteatonmyasthenicsyndromeacasereport
AT shiqiang autonomicdysfunctiondetectedbyskinsympatheticresponseinlamberteatonmyasthenicsyndromeacasereport