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
80-86Déclaration 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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