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Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome?
Morvan syndrome (MoS) is typically characterized by neuromyotonia, sleep dysfunction, dysautonomia, and cognitive dysfunction. However, MoS patients with mild peripheral nerve hyperexcitability (PNH) or encephalopathy features have been described. A 46-year-old woman presented with a 2-month history...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
S. Karger AG
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6739709/ https://www.ncbi.nlm.nih.gov/pubmed/31543789 http://dx.doi.org/10.1159/000497817 |
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author | Bakırcıoğlu-Duman, Ezgi Acar, Zeynep Benbir, Gülçin Yüceer, Hande Acar, Hürtan Baştan, Birgül Petek-Balcı, Belgin Karadeniz, Derya Çokar, Özlem Tüzün, Erdem |
author_facet | Bakırcıoğlu-Duman, Ezgi Acar, Zeynep Benbir, Gülçin Yüceer, Hande Acar, Hürtan Baştan, Birgül Petek-Balcı, Belgin Karadeniz, Derya Çokar, Özlem Tüzün, Erdem |
author_sort | Bakırcıoğlu-Duman, Ezgi |
collection | PubMed |
description | Morvan syndrome (MoS) is typically characterized by neuromyotonia, sleep dysfunction, dysautonomia, and cognitive dysfunction. However, MoS patients with mild peripheral nerve hyperexcitability (PNH) or encephalopathy features have been described. A 46-year-old woman presented with a 2-month history of constipation, hyperhidrosis, and insomnia. Neurologic examination revealed muscle twitching and needle electromyography showed myokymic discharges in all limbs. No clinical or electrophysiological features of neuromyotonia were present. Although the patient denied any cognitive symptoms, neuropsychological assessment revealed executive dysfunction, while other cognitive domains were preserved. Cranial and spinal MRIs were unrevealing and tumor investigation proved negative. Polysomnography examination revealed total insomnia, which was partially reversed upon immune-modulatory therapy. Investigation of a broad panel of antibodies revealed serum leucine-rich glioma inactivated protein 1 and contactin-associated protein 2 antibodies. The features of this case indicate that the presentation of PNH syndromes may show significant variability and that MoS patients may not necessarily exhibit full-scale PNH and encephalopathy symptoms. |
format | Online Article Text |
id | pubmed-6739709 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | S. Karger AG |
record_format | MEDLINE/PubMed |
spelling | pubmed-67397092019-09-22 Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome? Bakırcıoğlu-Duman, Ezgi Acar, Zeynep Benbir, Gülçin Yüceer, Hande Acar, Hürtan Baştan, Birgül Petek-Balcı, Belgin Karadeniz, Derya Çokar, Özlem Tüzün, Erdem Case Rep Neurol Case Report Morvan syndrome (MoS) is typically characterized by neuromyotonia, sleep dysfunction, dysautonomia, and cognitive dysfunction. However, MoS patients with mild peripheral nerve hyperexcitability (PNH) or encephalopathy features have been described. A 46-year-old woman presented with a 2-month history of constipation, hyperhidrosis, and insomnia. Neurologic examination revealed muscle twitching and needle electromyography showed myokymic discharges in all limbs. No clinical or electrophysiological features of neuromyotonia were present. Although the patient denied any cognitive symptoms, neuropsychological assessment revealed executive dysfunction, while other cognitive domains were preserved. Cranial and spinal MRIs were unrevealing and tumor investigation proved negative. Polysomnography examination revealed total insomnia, which was partially reversed upon immune-modulatory therapy. Investigation of a broad panel of antibodies revealed serum leucine-rich glioma inactivated protein 1 and contactin-associated protein 2 antibodies. The features of this case indicate that the presentation of PNH syndromes may show significant variability and that MoS patients may not necessarily exhibit full-scale PNH and encephalopathy symptoms. S. Karger AG 2019-02-28 /pmc/articles/PMC6739709/ /pubmed/31543789 http://dx.doi.org/10.1159/000497817 Text en Copyright © 2019 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission. |
spellingShingle | Case Report Bakırcıoğlu-Duman, Ezgi Acar, Zeynep Benbir, Gülçin Yüceer, Hande Acar, Hürtan Baştan, Birgül Petek-Balcı, Belgin Karadeniz, Derya Çokar, Özlem Tüzün, Erdem Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome? |
title | Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome? |
title_full | Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome? |
title_fullStr | Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome? |
title_full_unstemmed | Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome? |
title_short | Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome? |
title_sort | insomnia and dysautonomia with contactin-associated protein 2 and leucine-rich glioma inactivated protein 1 antibodies: a “forme fruste” of morvan syndrome? |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6739709/ https://www.ncbi.nlm.nih.gov/pubmed/31543789 http://dx.doi.org/10.1159/000497817 |
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