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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...

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Autores principales: Bakırcıoğlu-Duman, Ezgi, Acar, Zeynep, Benbir, Gülçin, Yüceer, Hande, Acar, Hürtan, Baştan, Birgül, Petek-Balcı, Belgin, Karadeniz, Derya, Çokar, Özlem, Tüzün, Erdem
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2019
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.
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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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