Literature DB >> 21906755

Morvan's syndrome associated with antibodies to multiple components of the voltage-gated potassium channel complex.

Panayiotis Loukaides1, Natasa Schiza, Philippa Pettingill, Lakis Palazis, Emmelia Vounou, Angela Vincent, Kleopas A Kleopa.   

Abstract

We describe a patient presenting with a combination of muscle fasciculations, paresthesias, hyperhidrosis, as well as insomnia, agitation and confusion. He went on to develop psychosis and respiratory failure requiring intensive care. Electromyography confirmed the presence of neuromyotonia and CSF showed mild pleocytosis. Routine testing for voltage-gated potassium channel complex (VGKC-complex) antibodies was highly positive, confirming the clinical diagnosis of Morvan's syndrome. The patient improved after treatment with intravenous immunoglobulin and methylprednisolone. Further investigation of the antigenic targets using immunohistochemistry and cell-based assays revealed that he had autoantibodies targeting Lgi1, Caspr2 and Contactin-2/Tag-1, all proteins known to be complexed with VGKC in peripheral nerves and CNS. This is the first case of Morvan's syndrome from Cyprus and illustrates the clinical features as well as the emerging complexity of antigenic targets involved in the pathogenesis.
Copyright © 2011 Elsevier B.V. All rights reserved.

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Year:  2011        PMID: 21906755     DOI: 10.1016/j.jns.2011.08.024

Source DB:  PubMed          Journal:  J Neurol Sci        ISSN: 0022-510X            Impact factor:   3.181


  8 in total

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Journal:  Front Immunol       Date:  2017-04-25       Impact factor: 7.561

2.  Paraneoplastic Morvan's syndrome following surgical treatment of recurrent thymoma: A case report.

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3.  Morvan Syndrome: A Case Report With Patient Narrative and Video.

Authors:  Mark Maskery; Suresh K Chhetri; Rejith Dayanandan; Claire Gall; Hedley C A Emsley
Journal:  Neurohospitalist       Date:  2016-01

Review 4.  Paraneoplastic neurological syndromes.

Authors:  F Leypoldt; K-P Wandinger
Journal:  Clin Exp Immunol       Date:  2014-03       Impact factor: 4.330

5.  Neuromyotonia with polyneuropathy, prominent psychoorganic syndrome, insomnia, and suicidal behavior without antibodies: a case report.

Authors:  Edvard Ehler; Alena Meleková
Journal:  J Med Case Rep       Date:  2015-05-06

6.  A Chinese female Morvan patient with LGI1 and CASPR2 antibodies: a case report.

Authors:  Li Zhang; Qiang Lu; Hong-Zhi Guan; Jun-Hua Mei; Hai-Tao Ren; Ming-Sheng Liu; Bin Peng; Li-Ying Cui
Journal:  BMC Neurol       Date:  2016-03-16       Impact factor: 2.474

7.  Untargeted screening for novel autoantibodies with prognostic value in first-episode psychosis.

Authors:  A Zandian; L Wingård; H Nilsson; E Sjöstedt; D X Johansson; D Just; C Hellström; M Uhlén; J M Schwenk; A Häggmark-Månberg; O Norbeck; B Owe-Larsson; P Nilsson; M A A Persson
Journal:  Transl Psychiatry       Date:  2017-07-25       Impact factor: 6.222

8.  Afterdischarges following M waves in patients with voltage-gated potassium channels antibodies.

Authors:  Jingwen Niu; Hongzhi Guan; Liying Cui; Yuzhou Guan; Mingsheng Liu
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  8 in total

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