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Anti-NMDA receptor encephalitis

MedGen UID:
458943
Concept ID:
C2986717
Disease or Syndrome
Synonym: Limbic encephalitis with NMDA receptor antibodies
SNOMED CT: Autoimmune encephalitis caused by N-methyl-D-aspartate receptor antibody (1259087007); Anti-NMDA receptor encephalitis (1259087007); Autoimmune encephalitis caused by N-methyl D-aspartate receptor antibody (452281000124106); Anti-NMDA (N-methyl D-Aspartate) receptor encephalitis (452281000124106)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0021081
Orphanet: ORPHA217253

Definition

A rare limbic encephalitis characterized by the presence of autoantibodies against NMDA receptors in serum and cerebrospinal fluid. It may be of paraneoplastic (most commonly associated with ovarian teratoma) or non-paraneoplastic origin and is life-threatening but potentially treatable. Patients present with acute behavioral change, psychosis, and catatonia, rapidly progressing to seizures, memory deficit, dyskinesias, speech problems, and autonomic and breathing dysregulation. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnti-NMDA receptor encephalitis

Professional guidelines

PubMed

Aneja S, Sharma S
Indian J Pediatr 2019 Jan;86(1):70-75. Epub 2018 Sep 19 doi: 10.1007/s12098-018-2775-0. PMID: 30232787
Gresa-Arribas N, Titulaer MJ, Torrents A, Aguilar E, McCracken L, Leypoldt F, Gleichman AJ, Balice-Gordon R, Rosenfeld MR, Lynch D, Graus F, Dalmau J
Lancet Neurol 2014 Feb;13(2):167-77. Epub 2013 Dec 18 doi: 10.1016/S1474-4422(13)70282-5. PMID: 24360484Free PMC Article
Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, Honig LS, Benseler SM, Kawachi I, Martinez-Hernandez E, Aguilar E, Gresa-Arribas N, Ryan-Florance N, Torrents A, Saiz A, Rosenfeld MR, Balice-Gordon R, Graus F, Dalmau J
Lancet Neurol 2013 Feb;12(2):157-65. Epub 2013 Jan 3 doi: 10.1016/S1474-4422(12)70310-1. PMID: 23290630Free PMC Article

Recent clinical studies

Etiology

Flanagan EP, Geschwind MD, Lopez-Chiriboga AS, Blackburn KM, Turaga S, Binks S, Zitser J, Gelfand JM, Day GS, Dunham SR, Rodenbeck SJ, Clardy SL, Solomon AJ, Pittock SJ, McKeon A, Dubey D, Zekeridou A, Toledano M, Turner LE, Vernino S, Irani SR
JAMA Neurol 2023 Jan 1;80(1):30-39. doi: 10.1001/jamaneurol.2022.4251. PMID: 36441519Free PMC Article
Muñoz-Lopetegi A, Graus F, Dalmau J, Santamaria J
Lancet Neurol 2020 Dec;19(12):1010-1022. doi: 10.1016/S1474-4422(20)30341-0. PMID: 33212053
Wang H
Curr Pharm Des 2020;25(43):4579-4588. doi: 10.2174/1381612825666191210155059. PMID: 31820697
Gresa-Arribas N, Titulaer MJ, Torrents A, Aguilar E, McCracken L, Leypoldt F, Gleichman AJ, Balice-Gordon R, Rosenfeld MR, Lynch D, Graus F, Dalmau J
Lancet Neurol 2014 Feb;13(2):167-77. Epub 2013 Dec 18 doi: 10.1016/S1474-4422(13)70282-5. PMID: 24360484Free PMC Article
Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, Honig LS, Benseler SM, Kawachi I, Martinez-Hernandez E, Aguilar E, Gresa-Arribas N, Ryan-Florance N, Torrents A, Saiz A, Rosenfeld MR, Balice-Gordon R, Graus F, Dalmau J
Lancet Neurol 2013 Feb;12(2):157-65. Epub 2013 Jan 3 doi: 10.1016/S1474-4422(12)70310-1. PMID: 23290630Free PMC Article

Diagnosis

Flanagan EP, Geschwind MD, Lopez-Chiriboga AS, Blackburn KM, Turaga S, Binks S, Zitser J, Gelfand JM, Day GS, Dunham SR, Rodenbeck SJ, Clardy SL, Solomon AJ, Pittock SJ, McKeon A, Dubey D, Zekeridou A, Toledano M, Turner LE, Vernino S, Irani SR
JAMA Neurol 2023 Jan 1;80(1):30-39. doi: 10.1001/jamaneurol.2022.4251. PMID: 36441519Free PMC Article
Dalmau J, Armangué T, Planagumà J, Radosevic M, Mannara F, Leypoldt F, Geis C, Lancaster E, Titulaer MJ, Rosenfeld MR, Graus F
Lancet Neurol 2019 Nov;18(11):1045-1057. Epub 2019 Jul 17 doi: 10.1016/S1474-4422(19)30244-3. PMID: 31326280
Aneja S, Sharma S
Indian J Pediatr 2019 Jan;86(1):70-75. Epub 2018 Sep 19 doi: 10.1007/s12098-018-2775-0. PMID: 30232787
Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, Honig LS, Benseler SM, Kawachi I, Martinez-Hernandez E, Aguilar E, Gresa-Arribas N, Ryan-Florance N, Torrents A, Saiz A, Rosenfeld MR, Balice-Gordon R, Graus F, Dalmau J
Lancet Neurol 2013 Feb;12(2):157-65. Epub 2013 Jan 3 doi: 10.1016/S1474-4422(12)70310-1. PMID: 23290630Free PMC Article
Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, Dessain SK, Rosenfeld MR, Balice-Gordon R, Lynch DR
Lancet Neurol 2008 Dec;7(12):1091-8. Epub 2008 Oct 11 doi: 10.1016/S1474-4422(08)70224-2. PMID: 18851928Free PMC Article

Therapy

Horie K, Barthélemy NR, Spina S, VandeVrede L, He Y, Paterson RW, Wright BA, Day GS, Davis AA, Karch CM, Seeley WW, Perrin RJ, Koppisetti RK, Shaikh F, Lago AL, Heuer HW, Ghoshal N, Gabelle A, Miller BL, Boxer AL, Bateman RJ, Sato C
Nat Med 2022 Dec;28(12):2547-2554. Epub 2022 Nov 24 doi: 10.1038/s41591-022-02075-9. PMID: 36424467Free PMC Article
Wang H
Curr Pharm Des 2020;25(43):4579-4588. doi: 10.2174/1381612825666191210155059. PMID: 31820697
Dalmau J, Armangué T, Planagumà J, Radosevic M, Mannara F, Leypoldt F, Geis C, Lancaster E, Titulaer MJ, Rosenfeld MR, Graus F
Lancet Neurol 2019 Nov;18(11):1045-1057. Epub 2019 Jul 17 doi: 10.1016/S1474-4422(19)30244-3. PMID: 31326280
Aneja S, Sharma S
Indian J Pediatr 2019 Jan;86(1):70-75. Epub 2018 Sep 19 doi: 10.1007/s12098-018-2775-0. PMID: 30232787
Jones KC, Benseler SM, Moharir M
Neuroimaging Clin N Am 2013 May;23(2):309-20. Epub 2013 Feb 28 doi: 10.1016/j.nic.2012.12.009. PMID: 23608692

Prognosis

Hiesgen J, Schutte C
S Afr Med J 2023 Mar 2;113(3):115. PMID: 36876357
Lin KL, Lin JJ
Biomed J 2020 Jun;43(3):251-258. Epub 2020 Apr 21 doi: 10.1016/j.bj.2020.04.002. PMID: 32330674Free PMC Article
Dalmau J, Armangué T, Planagumà J, Radosevic M, Mannara F, Leypoldt F, Geis C, Lancaster E, Titulaer MJ, Rosenfeld MR, Graus F
Lancet Neurol 2019 Nov;18(11):1045-1057. Epub 2019 Jul 17 doi: 10.1016/S1474-4422(19)30244-3. PMID: 31326280
Kayser MS, Dalmau J
Schizophr Res 2016 Sep;176(1):36-40. Epub 2014 Oct 25 doi: 10.1016/j.schres.2014.10.007. PMID: 25458857Free PMC Article
Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, Honig LS, Benseler SM, Kawachi I, Martinez-Hernandez E, Aguilar E, Gresa-Arribas N, Ryan-Florance N, Torrents A, Saiz A, Rosenfeld MR, Balice-Gordon R, Graus F, Dalmau J
Lancet Neurol 2013 Feb;12(2):157-65. Epub 2013 Jan 3 doi: 10.1016/S1474-4422(12)70310-1. PMID: 23290630Free PMC Article

Clinical prediction guides

Hiesgen J, Schutte C
S Afr Med J 2023 Mar 2;113(3):115. PMID: 36876357
Ma X, Lu Y, Peng F, Wang Y, Sun X, Luo W, Shen S, Liu Z, Kermode AG, Qiu W, Shu Y
Neurol Sci 2022 Jun;43(6):3893-3899. Epub 2022 Jan 18 doi: 10.1007/s10072-021-05838-3. PMID: 35041115
Gresa-Arribas N, Titulaer MJ, Torrents A, Aguilar E, McCracken L, Leypoldt F, Gleichman AJ, Balice-Gordon R, Rosenfeld MR, Lynch D, Graus F, Dalmau J
Lancet Neurol 2014 Feb;13(2):167-77. Epub 2013 Dec 18 doi: 10.1016/S1474-4422(13)70282-5. PMID: 24360484Free PMC Article
Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T, Honig LS, Benseler SM, Kawachi I, Martinez-Hernandez E, Aguilar E, Gresa-Arribas N, Ryan-Florance N, Torrents A, Saiz A, Rosenfeld MR, Balice-Gordon R, Graus F, Dalmau J
Lancet Neurol 2013 Feb;12(2):157-65. Epub 2013 Jan 3 doi: 10.1016/S1474-4422(12)70310-1. PMID: 23290630Free PMC Article
Peery HE, Day GS, Dunn S, Fritzler MJ, Prüss H, De Souza C, Doja A, Mossman K, Resch L, Xia C, Sakic B, Belbeck L, Foster WG
Autoimmun Rev 2012 Oct;11(12):863-72. Epub 2012 Mar 11 doi: 10.1016/j.autrev.2012.03.001. PMID: 22440397

Recent systematic reviews

Wang H
Front Immunol 2022;13:825103. Epub 2022 Mar 22 doi: 10.3389/fimmu.2022.825103. PMID: 35392089Free PMC Article
Giné-Servén E, Serra-Mestres J, Martinez-Ramirez M, Boix-Quintana E, Davi-Loscos E, Guanyabens N, Casado V, Muriana D, Torres-Rivas C, Cuevas-Esteban J, Labad J
Gen Hosp Psychiatry 2022 Jan-Feb;74:71-77. Epub 2021 Dec 3 doi: 10.1016/j.genhosppsych.2021.11.006. PMID: 34929551
Freund B, Ritzl EK
J Neuroimmunol 2019 Jul 15;332:64-68. Epub 2019 Mar 18 doi: 10.1016/j.jneuroim.2019.03.010. PMID: 30959339
Bacchi S, Franke K, Wewegama D, Needham E, Patel S, Menon D
J Clin Neurosci 2018 Jun;52:54-59. Epub 2018 Mar 28 doi: 10.1016/j.jocn.2018.03.026. PMID: 29605275
Wang H
Front Biosci (Landmark Ed) 2016 Jan 1;21(3):651-63. doi: 10.2741/4412. PMID: 26709797

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