Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A "Forme Fruste" of Morvan Syndrome?

Contactin-associated protein 2 Hyperexcitability Insomnia Leucine-rich glioma inactivated protein 1 Morvan syndrome Myokymia

Journal

Case reports in neurology
ISSN: 1662-680X
Titre abrégé: Case Rep Neurol
Pays: Switzerland
ID NLM: 101517693

Informations de publication

Date de publication:
Historique:
received: 17 01 2019
accepted: 08 02 2019
entrez: 24 9 2019
pubmed: 24 9 2019
medline: 24 9 2019
Statut: epublish

Résumé

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.

Identifiants

pubmed: 31543789
doi: 10.1159/000497817
pii: crn-0011-0080
pmc: PMC6739709
doi:

Types de publication

Case Reports

Langues

eng

Pagination

80-86

Déclaration de conflit d'intérêts

The authors declare that they have no conflicts of interest. No funds were received for this work.

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Auteurs

Ezgi Bakırcıoğlu-Duman (E)

Clinic of Neurology, Haseki Training and Research Hospital, Istanbul, Turkey.

Zeynep Acar (Z)

Clinic of Neurology, Haseki Training and Research Hospital, Istanbul, Turkey.

Gülçin Benbir (G)

Department of Neurology, Cerrahpaşa Faculty of Medicine, Istanbul University-Cerrahpaşa, Istanbul, Turkey.

Hande Yüceer (H)

Department of Neuroscience, Aziz Sancar Institute of Experimental Medical Research, Istanbul University, Istanbul, Turkey.

Hürtan Acar (H)

Clinic of Neurology, Haseki Training and Research Hospital, Istanbul, Turkey.

Birgül Baştan (B)

Clinic of Neurology, Haseki Training and Research Hospital, Istanbul, Turkey.

Belgin Petek-Balcı (B)

Clinic of Neurology, Haseki Training and Research Hospital, Istanbul, Turkey.

Derya Karadeniz (D)

Department of Neurology, Cerrahpaşa Faculty of Medicine, Istanbul University-Cerrahpaşa, Istanbul, Turkey.

Özlem Çokar (Ö)

Clinic of Neurology, Haseki Training and Research Hospital, Istanbul, Turkey.

Erdem Tüzün (E)

Department of Neuroscience, Aziz Sancar Institute of Experimental Medical Research, Istanbul University, Istanbul, Turkey.

Classifications MeSH