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Clinical features of seronegative, but CSF antibody-positive, anti-NMDA receptor encephalitis | Neurology Neuroimmunology & Neuroinflammation

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Anti-NMDA Receptor Encephalitis Foundation Newsletter

Anti-NMDA Receptor Encephalitis Foundation Newsletter | AntiNMDA | Scoop.it
A Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis Triggered by Herpes Simplex Virus Encephalitis

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Frontiers | Hello from the Other Side: How Autoantibodies Circumvent the Blood–Brain Barrier in Autoimmune Encephalitis | Immunology

Antibodies against neuronal receptors and synaptic proteins are associated with autoimmune encephalitides (AE) that produce movement and psychiatric disorders. In order to exert their pathological effects on neural circuits, autoantibodies against central nervous system (CNS) targets must gain access to the brain and spinal cord by crossing the blood-brain barrier (BBB), a tightly regulated gateway formed by endothelial cells lining CNS blood vessels. To date, the pathogenic mechanisms that underlie autoantibody-triggered encephalitic syndromes are poorly understood, and how autoantibodies breach the barrier remains obscure for almost all AE syndromes. The relative importance of cellular versus humoral immune mechanisms for disease pathogenesis also remains largely unexplored. Here we review the proposed triggers for various autoimmune encephalopathies and their animal models, as well as basic structural features of the BBB and how they differ among various CNS regions, a feature that likely underlies some regional aspects of autoimmune encephalitis pathogenesis. We then discuss the routes that antibodies and immune cells employ to enter the CNS and their implications for AE. Finally, we explore future therapeutic strategies that may either preserve or restore barrier function and thereby limit immune cell and autoantibody infiltration into the CNS. Recent mechanistic insights into CNS autoantibody entry indicate promising future directions for therapeutic intervention beyond current, short-lived therapies that eliminate circulating autoantibodies.
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A clinical approach to diagnosis of autoimmune encephalitis

Encephalitis is a severe inflammatory disorder of the brain with many possible causes
and a complex differential diagnosis. Advances in autoimmune encephalitis research
in the past 10 years have led to the identification of new syndromes and biomarkers
that have transformed the diagnostic approach to these disorders. However, existing
criteria for autoimmune encephalitis are too reliant on antibody testing and response
to immunotherapy, which might delay the diagnosis. We reviewed the literature and
gathered the experience of a team of experts with the aims of developing a practical,
syndrome-based diagnostic approach to autoimmune encephalitis and providing guidelines
to navigate through the differential diagnosis.
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My mysterious lost month of madness

My mysterious lost month of madness | AntiNMDA | Scoop.it
It was a cold March day as I walked to work from my Hell’s Kitchen studio. The weather was clear, people were out in their coats and scarves
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