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Related Concept Videos

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Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
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Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
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Arboviral encephalitis refers to brain inflammation caused by arthropod-borne viruses, particularly those transmitted through mosquito vectors. Among these, West Nile virus (WNV), a member of the Flaviviridae family, is a significant public health concern. WNV is an enveloped, positive-sense, single-stranded RNA virus. Human infection typically begins when an infected mosquito introduces the virus into the dermis during feeding. The primary transmission cycle involves birds as amplifying hosts...
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Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
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Autoimmune encephalopathies.

Frank Leypoldt1, Thaís Armangue, Josep Dalmau

  • 1Service of Neurology, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute (IDIBAPS), University of Barcelona, Barcelona, Spain; Institute of Clinical Chemistry, Neuroimmunology Unit and Department of Neurology, University Medical Center Schleswig-Holstein Campus, Kiel, Germany.

Annals of the New York Academy of Sciences
|October 16, 2014
PubMed
Summary
This summary is machine-generated.

Recent discoveries reveal novel autoimmune encephalitides targeting cell-surface proteins. This review details 11 disorders, their symptoms, antigens, and diagnostic challenges, highlighting advances in understanding brain autoimmunity.

Keywords:
anti-NMDAR antibodiesautoimmune encephalitislimbic encephalitispsychosistreatment

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Area of Science:

  • Neuroimmunology
  • Neurology
  • Cellular and Molecular Neuroscience

Background:

  • Autoimmune encephalitis diagnosis and treatment paradigms have evolved significantly due to novel antibody discoveries.
  • Previously unrecognized or mischaracterized neurological disorders are now understood as autoimmune conditions targeting neural proteins.

Purpose of the Study:

  • To review the discovery, clinical presentation, and target antigens of 11 autoimmune encephalitic disorders.
  • To provide a clinical perspective on grouping these disorders by syndrome.
  • To discuss diagnostic limitations and underlying pathogenic mechanisms.

Main Methods:

  • Clinical review of 11 autoimmune encephalitic disorders.
  • Analysis of target antigens, including cell-surface and synaptic proteins.
  • Examination of cellular and synaptic mechanisms of antibody pathogenicity.

Main Results:

  • Detailed description of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, limbic encephalitis subtypes, stiff-person spectrum disorders, and other encephalitides.
  • Introduction of a novel encephalopathy linked to IgLON5 autoimmunity and sleep dysfunction.
  • Identification of herpes simplex encephalitis as a trigger for synaptic autoimmunity and overlapping syndromes.

Conclusions:

  • Autoimmune encephalitis encompasses a growing spectrum of disorders with diverse clinical presentations and specific autoantibody targets.
  • Understanding antibody pathogenicity and diagnostic caveats is crucial for effective management.
  • Emerging research links infections and neurodegeneration to autoimmune processes in the brain.