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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.