U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Nudelman KNH, Jackson T, Rumbaugh M, Eloyan A, Abreu M, Dage JL, Snoddy C, Faber KM, Foroud T, Hammers DB; DIAN/DIAN-TU Clinical/Genetics Committee, Taurone A, Thangarajah M, Aisen P, Beckett L, Kramer J, Koeppe R, Kukull WA, Murray ME, Toga AW, Vemuri P, Atri A, Day GS, Duara R, Graff-Radford NR, Honig LS, Jones DT, Masdeu JC, Mendez M, Musiek E, Onyike CU, Riddle M, Rogalski E, Salloway S, Sha SJ, Turner RS, Wingo TS, Wolk DA, Carrillo MC, Dickerson BC, Rabinovici GD, Apostolova LG; LEADS Consortium
Alzheimers Dement 2023 Nov;19 Suppl 9(Suppl 9):S64-S73. Epub 2023 Oct 6 doi: 10.1002/alz.13482. PMID: 37801072Free PMC Article
Cho H, Mundada NS, Apostolova LG, Carrillo MC, Shankar R, Amuiri AN, Zeltzer E, Windon CC, Soleimani-Meigooni DN, Tanner JA, Heath CL, Lesman-Segev OH, Aisen P, Eloyan A, Lee HS, Hammers DB, Kirby K, Dage JL, Fagan A, Foroud T, Grinberg LT, Jack CR, Kramer J, Kukull WA, Murray ME, Nudelman K, Toga A, Vemuri P, Atri A, Day GS, Duara R, Graff-Radford NR, Honig LS, Jones DT, Masdeu J, Mendez M, Musiek E, Onyike CU, Riddle M, Rogalski EJ, Salloway S, Sha S, Turner RS, Wingo TS, Wolk DA, Koeppe R, Iaccarino L, Dickerson BC, La Joie R, Rabinovici GD; LEADS Consortium
Alzheimers Dement 2023 Nov;19 Suppl 9(Suppl 9):S98-S114. Epub 2023 Sep 10 doi: 10.1002/alz.13453. PMID: 37690109Free PMC Article
Dage JL, Eloyan A, Thangarajah M, Hammers DB, Fagan AM, Gray JD, Schindler SE, Snoddy C, Nudelman KNH, Faber KM, Foroud T, Aisen P, Griffin P, Grinberg LT, Iaccarino L, Kirby K, Kramer J, Koeppe R, Kukull WA, La Joie R, Mundada NS, Murray ME, Rumbaugh M, Soleimani-Meigooni DN, Toga AW, Touroutoglou A, Vemuri P, Atri A, Beckett LA, Day GS, Graff-Radford NR, Duara R, Honig LS, Jones DT, Masdeu JC, Mendez MF, Musiek E, Onyike CU, Riddle M, Rogalski E, Salloway S, Sha SJ, Turner RS, Wingo TS, Wolk DA, Womack KB, Carrillo MC, Dickerson BC, Rabinovici GD, Apostolova LG; LEADS Consortium
Alzheimers Dement 2023 Nov;19 Suppl 9(Suppl 9):S115-S125. Epub 2023 Jul 25 doi: 10.1002/alz.13399. PMID: 37491668Free PMC Article
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
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
Leypoldt F, Wandinger KP
Clin Exp Immunol 2014 Mar;175(3):336-48. doi: 10.1111/cei.12185. PMID: 23937626Free 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
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

Wang G, Li Y, Xiong C, Benzinger TLS, Gordon BA, Hassenstab J, Aschenbrenner AJ, McDade E, Clifford DB, Libre-Guerra JJ, Shi X, Mummery CJ, van Dyck CH, Lah JJ, Honig LS, Day G, Ringman JM, Brooks WS, Fox NC, Suzuki K, Levin J, Jucker M, Delmar P, Bittner T, Bateman RJ; DIAN‐TU Study Team
Alzheimers Dement 2024 Apr;20(4):2698-2706. Epub 2024 Feb 23 doi: 10.1002/alz.13735. PMID: 38400532Free PMC Article
Dage JL, Eloyan A, Thangarajah M, Hammers DB, Fagan AM, Gray JD, Schindler SE, Snoddy C, Nudelman KNH, Faber KM, Foroud T, Aisen P, Griffin P, Grinberg LT, Iaccarino L, Kirby K, Kramer J, Koeppe R, Kukull WA, La Joie R, Mundada NS, Murray ME, Rumbaugh M, Soleimani-Meigooni DN, Toga AW, Touroutoglou A, Vemuri P, Atri A, Beckett LA, Day GS, Graff-Radford NR, Duara R, Honig LS, Jones DT, Masdeu JC, Mendez MF, Musiek E, Onyike CU, Riddle M, Rogalski E, Salloway S, Sha SJ, Turner RS, Wingo TS, Wolk DA, Womack KB, Carrillo MC, Dickerson BC, Rabinovici GD, Apostolova LG; LEADS Consortium
Alzheimers Dement 2023 Nov;19 Suppl 9(Suppl 9):S115-S125. Epub 2023 Jul 25 doi: 10.1002/alz.13399. PMID: 37491668Free PMC Article
Chetram DK, Pan K, Elfasi A, Markham M
BMJ Case Rep 2021 Nov 19;14(11) doi: 10.1136/bcr-2021-244637. PMID: 34799390Free 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

Prognosis

Hiesgen J, Schutte C
S Afr Med J 2023 Mar 2;113(3):115. PMID: 36876357
Komagamine T, Kokubun N, Hirata K
Hist Psychiatry 2020 Mar;31(1):55-66. Epub 2019 Sep 20 doi: 10.1177/0957154X19877145. PMID: 31538814
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

Nudelman KNH, Jackson T, Rumbaugh M, Eloyan A, Abreu M, Dage JL, Snoddy C, Faber KM, Foroud T, Hammers DB; DIAN/DIAN-TU Clinical/Genetics Committee, Taurone A, Thangarajah M, Aisen P, Beckett L, Kramer J, Koeppe R, Kukull WA, Murray ME, Toga AW, Vemuri P, Atri A, Day GS, Duara R, Graff-Radford NR, Honig LS, Jones DT, Masdeu JC, Mendez M, Musiek E, Onyike CU, Riddle M, Rogalski E, Salloway S, Sha SJ, Turner RS, Wingo TS, Wolk DA, Carrillo MC, Dickerson BC, Rabinovici GD, Apostolova LG; LEADS Consortium
Alzheimers Dement 2023 Nov;19 Suppl 9(Suppl 9):S64-S73. Epub 2023 Oct 6 doi: 10.1002/alz.13482. PMID: 37801072Free PMC Article
Hiesgen J, Schutte C
S Afr Med J 2023 Mar 2;113(3):115. PMID: 36876357
Day GS, Gordon BA, McCullough A, Bucelli RC, Perrin RJ, Benzinger TLS, Ances BM
Ann Clin Transl Neurol 2021 Feb;8(2):491-497. Epub 2021 Jan 7 doi: 10.1002/acn3.51297. PMID: 33410601Free PMC Article
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 systematic reviews

Vasilevska V, Guest PC, Szardenings M, Benros ME, Steiner J
Transl Psychiatry 2024 Mar 8;14(1):139. doi: 10.1038/s41398-024-02831-0. PMID: 38459000Free PMC Article
Wang H
Front Immunol 2022;13:825103. Epub 2022 Mar 22 doi: 10.3389/fimmu.2022.825103. PMID: 35392089Free PMC Article
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...