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Immunotherapy responsive startle with antibodies to voltage gated potassium channels. 2009

Carlo Antozzi, and Simona Binelli, and Carolina Frassoni, and Claudia Ciano, and Angela Vincent, and Francesca Andreetta, and Ferruccio Panzica, and Silvana Franceschetti, and Paolo Confalonieri, and Renato Mantegazza
Myopathology and Immunology Unit, Neurological Institute Foundation "C Besta", Milan, Italy.

Antibodies to potassium channels (VGKC-Ab) were first associated with acquired neuromyotonia and its variant with CNS involvement, Morvan's syndrome. Recently, VGKC-Ab were found in patients with non-paraneoplastic limbic encephalitis (LE), characterised by personality changes, seizures and memory impairment. These patients may respond to immunotherapies. Thus the association of VGKC-Ab and non-paraneoplastic LE established the concept of a potentially reversible autoimmune encephalopathy. We describe a patient with startle syndrome and VGKC-Ab, without neuromyotonia or LE, who responded dramatically to plasma exchange (PE) and immunosuppression, adding to the spectrum of disorders associated with VGKC-Ab.

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