Journal
YEAR IN NEUROLOGY AND PSYCHIATRY
Volume 1338, Issue -, Pages 94-114Publisher
BLACKWELL SCIENCE PUBL
DOI: 10.1111/nyas.12553
Keywords
autoimmune encephalitis; limbic encephalitis; anti-NMDAR antibodies; psychosis; treatment
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Funding
- ICREA Funding Source: Custom
- NATIONAL INSTITUTE OF MENTAL HEALTH [R01MH094741] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS077851, RC1NS068204] Funding Source: NIH RePORTER
- NIMH NIH HHS [R01 MH094741] Funding Source: Medline
- NINDS NIH HHS [R01 NS077851, RC1 NS068204] Funding Source: Medline
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Over the past 10 years, the continual discovery of novel forms of encephalitis associated with antibodies to cell-surface or synaptic proteins has changed the paradigms for diagnosing and treating disorders that were previously unknown or mischaracterized. We review here the process of discovery, the symptoms, and the target antigens of 11 autoimmune encephalitic disorders, grouped by syndromes and approached from a clinical perspective. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, several subtypes of limbic encephalitis, stiff-person spectrum disorders, and other autoimmune encephalitides that result in psychosis, seizures, or abnormal movements are described in detail. We include a novel encephalopathy with prominent sleep dysfunction that provides an intriguing link between chronic neurodegeneration and cell-surface autoimmunity (IgLON5). Some of the caveats of limited serum testing are outlined. In addition, we review the underlying cellular and synaptic mechanisms that for some disorders confirm the antibody pathogenicity. The multidisciplinary impact of autoimmune encephalitis has been expanded recently by the discovery that herpes simplex encephalitis is a robust trigger of synaptic autoimmunity, and that some patients may develop overlapping syndromes, including anti-NMDAR encephalitis and neuromyelitis optica or other demyelinating diseases.
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