anti-NMDA receptor encephalitis; NMDAR Ab encephalitis
Jump to navigation
Jump to search
Etiology
- associated with anti-NMDA receptor antibodies
- most commonly associated with bilateral ovarian teratoma
Epidemiology
- 80% women
- most commonly occurs age 2-40 years[4]
Pathology
- antibody against NMDA receptor (cell surface receptor)
- neurons expressing this receptor remain intact[4]
Clinical manifestations
- onset may be heralded by flu-like syndrome
- evolution over weeks to months
- altered mental status
- choreoathetosis[1]
- intermittent muscle rigidity
- involuntary jerking movements of the arms & legs[4]
- seizures[2]
- autonomic instability[1]
- palpitations, sinus tachycardia
- intermittent fever
- hypertension
- no focal neurologic deficits, no nuchal rigidity[4]
Laboratory
- CSF analysis
- normal or mild CSF lymphocyte pleocytosis
- normal or mildly elevated CSF protein
- anti-NMDA receptor antibody in CSF & serum
Diagnostic procedures
- pelvic ultrasound for ovarian teratoma
- ovarian teratomas implicated in NMDAR Ab encephalitis
Radiology
- neuroimaging
- MRI may show FLAIR signals in one or both temporal lobes
Differential diagnosis
Management
- empiric IV acyclovir for Herpes simplex encephalitis until diagnosis is clarified[1]
- IV vancomycin, ceftriaxone, & acyclovir used in ref[4]
- ovariectomy if teratoma; full remission has resulted[4]
- immunosuppressive therapy:
- combination of plasmapheresis, intravenous gamma-globulin & glucocorticoids[4]
- addition of rituximab or cyclophosphamide reserved for refractory cases[4]
- combination of plasmapheresis, intravenous gamma-globulin & glucocorticoids[4]
Prognosis:
- full recovery after surgical removal of teratoma may take months[4]
- 75% of patients recover either completely or substantially[4]
More general terms
Additional terms
References
- ↑ Jump up to: 1.0 1.1 1.2 1.3 Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18. American College of Physicians, Philadelphia 2012, 2015, 2018.
- ↑ Jump up to: 2.0 2.1 Dalmau J, Gleichman AJ, Hughes EG et al Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008 Dec;7(12):1091-8 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18851928
- ↑ Titulaer MJ, McCracken L, Gabilondo I et al Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013 Feb;12(2):157-65. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23290630 Free PMC Article
- ↑ Jump up to: 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 4.12 4.13 4.14 Mathai SK, Josephson SA, Badlam J et al Scratching Below the Surface. N Engl J Med 2016; 375:2188-2193. December 1, 2016 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27959764 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcps1603154
- ↑ Dalmau J, Graus F. Antibody-Mediated Encephalitis. N Engl J Med. 2018 Mar 1;378(9):840-851. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29490181
- ↑ 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