Rare disease
Immunotherapy responsive startle with antibodies to voltage gated potassium channels
1 Myopathology and Immunology Unit, Neurological Institute Foundation "C Besta", Milan, Italy
2 Clinical Neurophysiology Unit, Neurological Institute Foundation "C Besta", Milan, Italy
3 Experimental Neurophysiology Unit, Neurological Institute Foundation "C Besta", Milan, Italy
4 Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford, UK
Correspondence to:
antozzi{at}istituto-besta.it
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.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
- Full Text
- Submit a response
- Alert me when this article is cited
- Alert me when eLetters are posted
- Alert me if a correction is posted
Services
- Email this link to a friend
- Similar articles in this journal
- Add article to my folders
- Download to citation manager
- Request Permissions
Citing Articles
Google Scholar
PubMed
Topic Collections
Bookmark with
Register for free content
The full text of all Editor's Choice articles and summaries of every article are free without registration
The full text of Images in ... articles are free to registered users
Only fellows can access the full text of case reports (apart from Editor's Choice) - become a fellow today, or encourage your institution to, so that together we can grow and develop this resource
Don't forget to sign up for content alerts so you keep up to date with all the case reports as they are published, and let us know what you think by commenting on the Editor's blog
