autoimmune encephalomyelitis
Jump to navigation
Jump to search
Etiology
- NMDA receptor autoantibody
- viruses can also induce an autoimmune response
- Herpes simplex virus encephalitis can trigger subsequent anti-NMDA receptor encephalitis
- viruses can also induce an autoimmune response
- LGI1 encephalitis
Epidemiology
- women < 35 years
Clinical manifestations
- subacute presentation (symptom duration > 5 days)
- memory or psychiatric manifestations
- behavioral changes, headaches, & fever followed by
- altered mental status, seizures, abnormal movements, & autonomic instability[3]
- no comorbidities
Laboratory
- CSF leukocyte count < 50/uL or CSF protein < 50 mg/dL
- NMDA receptor IgG in CSF
- LGI1 Ab in CSF
- see ARUP consult[1]
Radiology
Differential diagnosis
- West Nile virus encephalitis
- normal MRI makes West Nile virus less likely
- insidious onset, positive nonspecific serum antibodies suggest other diagnosis[2]
Management
- intravenous glucocorticoids, intravenous immune globulin
- in some cases, plasmapheresis & rituximab
- removal of ovarian tumor (if present)
More general terms
More specific terms
- anti-NMDA receptor encephalitis; NMDAR Ab encephalitis
- experimental autoimmune encephalomyelitis
- paraneoplastic encephalomyelitis sensory neuropathy
References
- ↑ 1.0 1.1 ARUP Consult: N-methyl-D-Aspartate (NMDA) type Glutamate Receptor Autoantibody Disorders Anti-NMDA-Receptor Encephalitis The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/n-methyl-d-aspartate-nmda-type-glutamate-receptor-autoantibody-disorders
Anti-NMDA Receptor (NR1) IgG Antibodies https://arupconsult.com/ati/anti-nmda-receptor-nr1-igg-antibodies - ↑ 2.0 2.1 Flanagan EP et al. Autoimmune encephalitis misdiagnosis in adults. JAMA Neurol 2022 Nov 28; [e-pub]. PMID: https://pubmed.ncbi.nlm.nih.gov/36441519 PMCID: PMC9706400 Free PMC article https://jamanetwork.com/journals/jamaneurology/fullarticle/2799083
- ↑ 3.0 3.1 3.2 Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025
- ↑ Granillo A, Le Marechal M, Diaz-Arias L, et al. Development and validation of a risk score to differentiate viral and autoimmune encephalitis in adults. Clin Infect Dis. 2023;76:e1294-e1301. PMID: https://pubmed.ncbi.nlm.nih.gov/36053949