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Voltage-Gated Potassium Channel Complex

Link PDF: Autoantibodies associated with diseases of the CNS: new developments and future challenges

Several CNS disorders associated with specifi c antibodies to ion channels, receptors, and other synaptic proteins have been recognised over the past 10 years, and can be often successfully treated with immunotherapies. Antibodies to components of voltage-gated potassium channel complexes (VGKCs), NMDA receptors (NMDARs), AMPA receptors (AMPARs), GABA type B receptors (GABABRs), and glycine receptors (GlyRs) can be identifi ed in patients and are associated with various clinical presentations, such as limbic encephalitis and complex and diff use encephalopathies. These diseases can be associated with tumours, but they are more often non-paraneoplastic, and antibody assays can help with diagnosis. The new specialty of immunotherapy-responsive CNS disorders is likely to expand further as more antibody targets are discovered. Recent fi ndings raise many questions about the classifi cation of these diseases, the relation between antibodies and specifi c clinical phenotypes, the relative pathological roles of serum and intrathecal antibodies, the mechanisms of autoantibody generation, and the development of optimum treatment strategies.

This article by Angela Vincent, Christian Bien, Sarosh Irani, and Patrick Waters talks about conditions of the CNS caused by autoantibodies. The full paper can be found at ResearchGate.net. This was posted to thread https://www.facebook.com/groups/251477975360/permalink/10154711712105361/ on 27th October, 2014 by G.D.

Copyright © The Lancet 2011 | Authors Angela Vincent, Christian Bien, Sarosh Irani, and Patric Waters

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

Link: Rethinking therapy decisions in autoimmune encephalopathy

Researchers say that diagnosis and management of encephalopathies may need to be rethought, as many patients who test negative for the relevant antibody still benefit from immunotherapy.

Of 48 patients with a diagnosis of probable autoimmune encephalopathy, only 21 (44%) individuals were positive for antibodies known to be associated with this type of encephalopathy. However, both antibody-positive and antibody-negative patients responded to immunotherapy, with complete recovery achieved in 20 (42%) children, after a mean follow-up period of 24 months.

The full article can be found at http://linkis.com/www.news-medical.net/oSP1E.

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

Link: Autoimmune Channelopathies of the Nervous System

Ion channels are complex transmembrane proteins that orchestrate the electrical signals necessary for normal function of excitable tissues, including the central nervous system, peripheral nerve, and both skeletal and cardiac muscle. Progress in molecular biology has allowed cloning and expression of genes that encode channel proteins, while comparable advances in biophysics, including patch-clamp electrophysiology and related techniques, have made the functional assessment of expressed proteins at the level of single channel molecules possible. The role of ion channel defects in the pathogenesis of numerous disorders has become increasingly apparent over the last two decades. Neurological channelopathies are frequently genetically determined but may also be acquired through autoimmune mechanisms. All of these autoimmune conditions can arise as paraneoplastic syndromes or independent from malignancies. The pathogenicity of autoantibodies to ion channels has been demonstrated in most of these conditions, and patients may respond well to immunotherapies that reduce the levels of the pathogenic autoantibodies. Autoimmune channelopathies may have a good prognosis, especially if diagnosed and treated early, and if they are non-paraneoplastic. This review focuses on clinical, pathophysiologic and therapeutic aspects of autoimmune ion channel disorders of the nervous system.

This link was posted by G.D. on 28th August, 2014. The full study can be found at http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3151600/.

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

Link: Autoantibodies to neuronal surface antigens in thyroid antibody-positive and -negative limbic encephalitis

Background : Thyroid antibodies (Thy-Abs) are frequently detected in various autoimmune disorders in coexistence with other systemic autoantibodies. In association with an encephalopathy, they are often taken as evidence of Hashimoto’s encephalitis (HE). However, the presence of Thy-Abs in a cohort of limbic encephalitis (LE) patients and their association with anti-neuronal autoimmunity has not been explored. Patients and Methods : We investigated thyroid and anti-neuronal antibodies in the sera of 24 LE patients without identified tumors by cell-based assay and radioimmunoassay and evaluated their clinical features. Results : There was a female predominance in Thy-Ab-positive LE patients. Five of the eight Thy-Ab-positive patients and six of the 16 Thy-Ab-negative patients had antibodies to voltage-gated potassium channel (VGKC), N-methyl-D-aspartate receptor (NMDAR) or undefined surface antigens on cultured hippocampal neurons. There were trends towards fewer VGKC antibodies (1/8 vs. 5/16, P = 0.159) and more NMDAR antibodies (2/8 vs. 1/16, P = 0.095) among the Thy-Ab-positive LE patients; antibodies to undefined surface antigens were only identified in Thy-Ab-positive patients (2/8 vs. 0/16, P = 0.018). There were no distinguishing clinical features between Thy-Ab-positive patients with and without neuronal antibodies. However, patients with anti-neuronal antibodies showed a better treatment response. Conclusion : Thy-Abs can be found in a high proportion of patients with non-paraneoplastic LE, often in association with antibodies to specific or as yet undefined neuronal surface antigens. These results suggest that acute idiopathic encephalitis patients with Thy-Abs should be closely monitored for ion-channel antibodies and it should not be assumed that they have HE.

 

The full study can be found at http://neurologyindia.com.

Copyright © Neurology India 2011

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

Link: The neurologic significance of celiac disease biomarkers

Objective:

To report neurologic phenotypes and their etiologies determined among 68 patients with either (1) celiac disease (CD) or (2) no CD, but gliadin antibody positivity (2002-2012).

 

The full text could not be found of this article, however the abstract and conclusions can be found at http://www.ncbi.nlm.nih.gov/pubmed/25261501.

© 2014 American Academy of Neurology

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

Link: Types Of Autoimmune Encephalitis

Autoimmune Encephalitis may be triggered by infection in which case the term “Post-infectious Encephalitis” is used. ADEM( Acute Disseminated Encephalomyelitis ) is a Post-infectious Encephalitis. The illness usually follows in the wake of a mild viral infection (such as those that cause rashes in childhood) or immunisations. Typically there is a delay of days to two to three weeks between the triggering infection and development of the Encephalitis.

This link will lead you to a variety of auto-immuned encephalopathies and more information about each.

 

Copyright © The Encephalitis Society

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

PDF Link: Autoantibodies Associated with Diseases of the CNS

Linked is a lengthy PDF article regarding multiple auto-immune diseases affecting the Central Nervous System.

Several CNS disorders associated with specifi c antibodies to ion channels, receptors, and other synaptic proteins have
been recognised over the past 10 years, and can be often successfully treated with immunotherapies. Antibodies to
components of voltage-gated potassium channel complexes (VGKCs), NMDA receptors (NMDARs), AMPA receptors
(AMPARs), GABA type B receptors (GABABRs), and glycine receptors (GlyRs) can be identifi ed in patients and are
associated with various clinical presentations, such as limbic encephalitis and complex and diff use encephalopathies.
These diseases can be associated with tumours, but they are more often non-paraneoplastic, and antibody assays can
help with diagnosis. The new specialty of immunotherapy-responsive CNS disorders is likely to expand further as
more antibody targets are discovered. Recent fi ndings raise many questions about the classifi cation of these diseases,
the relation between antibodies and specifi c clinical phenotypes, the relative pathological roles of serum and intrathecal
antibodies, the mechanisms of autoantibody generation, and the development of optimum treatment strategies.

 

The PDF can be found at Google Articles, published by www.thelancet.com in their August 2011 journal, Volume 10.

Copyright © 2011 The Lancet

 

 

 

 

 

This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.

Link: Autoimmune Encephalopathy

This MedScape article in “seminars in neurology”, written by Eoin P. Flanagan, M.B.B.Ch., Richard J. Caselli, M.D. for the Department of Neurology, Mayo Clinic, Rochester, Minnesota; and Department of Neurology, Mayo Clinic, Scottsdale, Arizona., and discusses non-paraneoplastic encephalopathy. It focuses on the global effects of auto-immune encephalopathy laboratory testing, imaging tools, anti-body testing, ruling out cancerous causes, and ruling out CJD, among other topics.

http://www.medscape.com/viewarticle/743774

The full text of this article is found at MedScape and was published in 2011. © 2011 Thieme Medical Publishers

 

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This website is not a substitute for independent professional advice. Nothing contained in this site is intended to be used as medical advice. No articles, personal accounts, or other content are intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professionals advice.