title: Immunotherapy responsive startle with antibodies to voltage gated potassium channels.
abstract: 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.
authors.affiliation: Myopathology and Immunology Unit, Neurological Institute Foundation "C Besta", Milan, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Antozzi
authors.forename: Simona
authors.initials: S
authors.lastname: Binelli
authors.forename: Carolina
authors.initials: C
authors.lastname: Frassoni
authors.forename: Claudia
authors.initials: C
authors.lastname: Ciano
authors.forename: Angela
authors.initials: A
authors.lastname: Vincent
authors.forename: Francesca
authors.initials: F
authors.lastname: Andreetta
authors.forename: Ferruccio
authors.initials: F
authors.lastname: Panzica
authors.forename: Silvana
authors.initials: S
authors.lastname: Franceschetti
authors.forename: Paolo
authors.initials: P
authors.lastname: Confalonieri
authors.forename: Renato
authors.initials: R
authors.lastname: Mantegazza