Article Text

Rare disease
Immunotherapy responsive startle with antibodies to voltage gated potassium channels
  1. Carlo Antozzi1,
  2. Simona Binelli2,
  3. Carolina Frassoni3,
  4. Claudia Ciano2,
  5. Angela Vincent4,
  6. Francesca Andreetta1,
  7. Ferruccio Panzica2,
  8. Silvana Franceschetti1,
  9. Paolo Confalonieri1,
  10. Renato Mantegazza1
  1. 1
    Myopathology and Immunology Unit, Neurological Institute Foundation “C Besta”, Milan, Italy
  2. 2
    Clinical Neurophysiology Unit, Neurological Institute Foundation “C Besta”, Milan, Italy
  3. 3
    Experimental Neurophysiology Unit, Neurological Institute Foundation “C Besta”, Milan, Italy
  4. 4
    Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford, UK
  1. antozzi{at}


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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  • Competing interests: None.