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.
Keywords
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Article info
Publication history
Published online: September 09, 2011
Accepted:
August 16,
2011
Received in revised form:
August 11,
2011
Received:
May 30,
2011
Identification
Copyright
© 2011 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
