Autoimmune encephalitis
| Acute disseminated encephalomyelitis
Summary
Autoimmune encephalitis refers to a group of conditions that occur when the body's immune system mistakenly attacks healthy brain cells, leading to inflammation of the brain. People with Autoimmune encephalitis may have various neurologic and/or psychiatric symptoms. Neurologic symptoms may include impaired memory and cognition, abnormal movements, seizures, and/or problems with balance, speech, or vision. Psychiatric symptoms may include psychosis, aggression, inappropriate sexual behaviors, panic attacks, compulsive behaviors, euphoria or fear. Symptoms may fluctuate, but often progress over days to a few weeks. Symptoms can progress to loss of consciousness or even coma. Autoimmune encephalitis may be associated with antibodies to proteins on the surface of nerve cells, or within nerve cells. Some of these proteins are involved in passing signals between nerve cells. In some cases it occurs in association with cancer (a paraneoplastic syndrome). Research regarding why specific antibodies attack the body's healthy cells is ongoing. Autoimmune encephalitis generally occurs sporadically, in people with no family history of the condition.
( Genetic and Rare Diseases Information Center, 2023)
Symptoms
Fever, headaches, irritability, fatigue, lethargy, malaise, unintended weight loss, nausea, and vomiting, vision loss in one or both eyes, muscle weakness even to the point of paralysis, difficulty coordinating voluntary muscle movements, confusion, stupor, delirium, and potentially coma.
(Global Autoimmune Institute, 2023)
Diagnostic Criteria
Pediatric
Adult
Diagnostic Tests
Adult
Pediatric
Human Leukocyte Antigen (HLA) Associations
Under Investigation
Tissue-Type or Cell-Type Attacked
Under Investigation
Attacking Immune Cell Type or Process
Under Investigation
Treatment Modality
Under Investigation
Managing Specialist(s)
Neurology
Rheumatology
Associated Infections
Under Investigation
Associated Toxins
Under Investigation
Research Authors
Under Investigation
Research Institutions
Under Investigation
Average Time from Symptom Onset to Diagnosis
Under Investigation
Last Updated
February 13, 2023
References
Autoimmune Encephalitis (AE) | Acute Disseminated Encephalomyelitis (ADEM). Global Autoimmune Institute. (2023). Retrieved February 10, 2023 from https://www.autoimmuneinstitute.org/acute-disseminated-encephalomyelitis/
Autoimmune encephalitis. National Institutes of Health, National Center for Advancing Translational Science, Genetic and Rare Diseases Information Center. (2023, February). Retrieved February 10, 2023 from https://rarediseases.info.nih.gov/diseases/11979/autoimmune-encephalitis
Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient. Tania Cellucci, Heather Van Mater, Francesc Graus, Eyal Muscal, William Gallentine, Marisa S. Klein-Gitelman, Susanne M. Benseler, Jennifer Frankovich, Mark P. Gorman, Keith Van Haren, JosepDalmau, Russell C. Dale. Neurol Neuroimmunol Neuroinflamm Mar 2020, 7 (2) e663; DOI:10.1212/NXI.0000000000000663.
Venkatesan A, Tunkel AR, Bloch KC, Lauring AS, Sejvar J, Bitnun A, Stahl JP, Mailles A, Drebot M, Rupprecht CE, Yoder J, Cope JR, Wilson MR, Whitley RJ, Sullivan J, Granerod J, Jones C, Eastwood K, Ward KN, Durrheim DN, Solbrig MV, Guo-Dong L, Glaser CA; International Encephalitis Consortium. Case definitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the international encephalitis consortium. Clin Infect Dis. 2013 Oct;57(8):1114-28. doi: 10.1093/cid/cit458. Epub 2013 Jul 15. PMID: 23861361; PMCID: PMC3783060.