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Morvan's syndrome associated with antibodies to multiple components of the voltage-gated potassium channel complex.
- Source :
-
Journal of the neurological sciences [J Neurol Sci] 2012 Jan 15; Vol. 312 (1-2), pp. 52-6. Date of Electronic Publication: 2011 Sep 08. - Publication Year :
- 2012
-
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.<br /> (Copyright © 2011 Elsevier B.V. All rights reserved.)
- Subjects :
- Aged
Autoantibodies biosynthesis
Contactin 2 immunology
Humans
Intracellular Signaling Peptides and Proteins
Isaacs Syndrome drug therapy
Male
Membrane Proteins immunology
Myokymia drug therapy
Nerve Tissue Proteins immunology
Proteins immunology
Autoantibodies blood
Isaacs Syndrome immunology
Myokymia immunology
Potassium Channels, Voltage-Gated immunology
Subjects
Details
- Language :
- English
- ISSN :
- 1878-5883
- Volume :
- 312
- Issue :
- 1-2
- Database :
- MEDLINE
- Journal :
- Journal of the neurological sciences
- Publication Type :
- Academic Journal
- Accession number :
- 21906755
- Full Text :
- https://doi.org/10.1016/j.jns.2011.08.024