Neuronal Surface Antibody-Mediated Autoimmune Encephalitis

Semin Neurol 2014; 34: 458-466DOI: 10.1055/s-0034-1390394In the past few years, many autoimmune encephalitides have been identified, with specific clinical syndromes and associated antibodies against neuronal surface antigens. There is compelling evidence that many of these antibodies are pathogenic and most of these encephalitides are highly responsive to immunotherapies. The clinical spectra of some of these antibody-mediated syndromes, especially those reported in only a few patients, are evolving. Others, such as anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, are well characterized. Diagnosis involves recognizing the specific syndromes and identifying the antibody in a patient's cerebrospinal fluid (CSF) and/or serum. These syndromes are associated with variable abnormalities in CSF, magnetic resonance imaging, and electroencephalography. Treatment is often multidisciplinary and should be focused upon neutralizing the effects of antibodies and eliminating their source. Overlapping disorders have been noted, with some patients having more than one neurologic autoimmune disease. In other patients, viral infections such as herpes simplex virus encephalitis trigger robust antineuronal autoimmune responses.[...]Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents  |  Abstract  |  Full text
Source: Seminars in Neurology - Category: Neurology Authors: Source Type: research

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An association between herpes simplex virus (HSV) encephalitis and NDMA receptor (NMDAR) encephalitis has been well described. Here, we report a rare case of HSV encephalitis occurring alongside autoimmune encephalitis with leucine-rich glioma inactivated 1 (LGI-1) and NMDAR antibodies.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, Encephalitis, Viral infections Clinical/Scientific Notes Source Type: research
nhold D Abstract Changes in levels of cytokines or soluble receptors in biological fluids may provide information on immunological pathomechanisms underlying the respective diseases. Here, we studied cytokine patterns of patients with autoimmune encephalitis (AE) before and after immunosuppressive treatment in order to identify possible biomarker candidates and to look for putatively involved pathomechanisms. We performed measurements in Cerebrospinal fluid (CSF) and serum of 7 patients suffering from AE with antibodies (ab) against Leucine-rich glioma-inactivated-protein 1 (LGI1) and 9 AE patients with Contactin-...
Source: Cytokine - Category: Molecular Biology Authors: Tags: Cytokine Source Type: research
ConclusionsIn appropriately selected patients, FDG-PET scans while in burst suppression may help dissect the underlying pathophysiologic cause of IIC findings observed on EEG and guide tailored therapy.
Source: Neurocritical Care - Category: Neurology Source Type: research
Abstract Autoimmune encephalitides, with an estimated incidence of 1.5 per million population per year, although described only 15 years ago, have already had a remarkable impact in neurology and paved the field to autoimmune neuropsychiatry. Many patients traditionally presented with aberrant behavior, especially of acute or subacute onset, and treated with anti-psychotic therapies, turn out to have a CNS autoimmune disease with pathogenic autoantibodies against synaptic antigens responding to immunotherapies. The review describes the clinical spectrum of these disorders, and the pathogenetic role of key autoanti...
Source: Journal of Autoimmunity - Category: Allergy & Immunology Authors: Tags: J Autoimmun Source Type: research
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Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, Encephalitis, Viral infections, Post-infectious, Status epilepticus Clinical/Scientific Notes Source Type: research
The Toll-like receptor 3 (TLR3) pathway is a key component of the innate immunity that prevents replication of viruses in the CNS. Inborn errors of this pathway (TLR3-pathway deficiency), which includes defects in the genes TLR3, UNC93B1, TRIF, TRAF3, TBK1 and IRF3, occur in 10% of patients with herpes simplex encephalitis (HSE),1,2 and about 66% of these patients develop relapses of HSE.1 A recent study showed that 27% of patients with HSE develop autoimmune encephalitis (AE) in the weeks or months ensuing the infection.3 It is unknown whether TLR3-pathway deficient patients can also develop AE post-HSE. Here we report a ...
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, Encephalitis, Viral infections, Post-infectious, Psychosis Clinical/Scientific Notes Source Type: research
Authors: Joubert B, Dalmau J Abstract Autoimmune encephalitides are autoimmune neurological disorders characterized by rapidly progressive central nervous system symptoms associated with specific auto-antibodies targeting neuronal cell-surface proteins. The clinical features of encephalitis are frequently preceded by symptoms suggesting an infectious process, and specific pathogens have been detected at the early phase of the disease in some patients, suggesting that it can be triggered by infections. Moreover, recent data have shown an association with specific HLA haplotypes, suggesting a genetic susceptibility t...
Source: Revue Neurologique - Category: Neurology Tags: Rev Neurol (Paris) Source Type: research
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Source: The Lancet Neurology - Category: Neurology Source Type: research
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Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
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Source: Current Opinion in Infectious Diseases - Category: Infectious Diseases Tags: CNS INFECTIONS: Edited by Adarsh Bhimraj Source Type: research
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