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Autoimmune limbic encephalitis

MedGen UID:
419645
Concept ID:
C2930824
Disease or Syndrome
Synonyms: ALE; autoimmune limbic encephalitis
 
Monarch Initiative: MONDO:0850097
Orphanet: ORPHA623615

Definition

A rare autoimmune encephalitis involving the mesial temporal lobes and clinically characterized by subacute onset (i. e. rapid progression of less than three months) of short-term memory deficits, seizures or psychiatric symptoms, such as behavioral changes, anxiety, depression, and psychosis. Further diagnostic criteria are bilateral abnormalities restricted to the mesial temporal lobes in brain MRI, cerebrospinal fluid pleocytosis and/or epileptic or slow-wave activity involving the temporal lobes in EEG, and reasonable exclusion of alternative causes. Paraneoplastic or non-paraneoplastic antibodies against neuronal antigens may be found in serum and/or cerebrospinal fluid. [from ORDO]

Professional guidelines

PubMed

Van Steenhoven RW, de Vries JM, Bruijstens AL, Paunovic M, Nagtzaam MM, Franken SC, Bastiaansen AE, De Bruijn MA, Van Sonderen A, Schreurs MWJ, Gardeniers M, Verdijk RM, Balvers RK, Sillevis Smitt PA, Neuteboom RF, Titulaer MJ
Neurol Neuroimmunol Neuroinflamm 2023 Nov;10(6) Epub 2023 Aug 15 doi: 10.1212/NXI.0000000000200148. PMID: 37582614Free PMC Article
Lee WJ, Lee ST, Byun JI, Sunwoo JS, Kim TJ, Lim JA, Moon J, Lee HS, Shin YW, Lee KJ, Kim S, Jung KH, Jung KY, Chu K, Lee SK
Neurology 2016 May 3;86(18):1683-91. Epub 2016 Apr 1 doi: 10.1212/WNL.0000000000002635. PMID: 27037228
da Rocha AJ, Nunes RH, Maia AC Jr, do Amaral LL
AJNR Am J Neuroradiol 2015 Dec;36(12):2196-205. Epub 2015 Sep 17 doi: 10.3174/ajnr.A4408. PMID: 26381566Free PMC Article

Recent clinical studies

Etiology

Mojžišová H, Krýsl D, Hanzalová J, Dargvainiene J, Wandinger KP, Leypoldt F, Elišák M, Marusič P
Neurol Neuroimmunol Neuroinflamm 2023 Nov;10(6) Epub 2023 Oct 25 doi: 10.1212/NXI.0000000000200170. PMID: 37879962Free PMC Article
Van Steenhoven RW, de Vries JM, Bruijstens AL, Paunovic M, Nagtzaam MM, Franken SC, Bastiaansen AE, De Bruijn MA, Van Sonderen A, Schreurs MWJ, Gardeniers M, Verdijk RM, Balvers RK, Sillevis Smitt PA, Neuteboom RF, Titulaer MJ
Neurol Neuroimmunol Neuroinflamm 2023 Nov;10(6) Epub 2023 Aug 15 doi: 10.1212/NXI.0000000000200148. PMID: 37582614Free PMC Article
Comperat L, Pegat A, Honnorat J, Joubert B
Curr Opin Neurol 2022 Oct 1;35(5):597-603. Epub 2022 Aug 19 doi: 10.1097/WCO.0000000000001104. PMID: 35989569
Lee WJ, Lee HS, Kim DY, Lee HS, Moon J, Park KI, Lee SK, Chu K, Lee ST
Brain 2022 Oct 21;145(10):3509-3521. doi: 10.1093/brain/awac166. PMID: 35512357
van Sonderen A, Thijs RD, Coenders EC, Jiskoot LC, Sanchez E, de Bruijn MA, van Coevorden-Hameete MH, Wirtz PW, Schreurs MW, Sillevis Smitt PA, Titulaer MJ
Neurology 2016 Oct 4;87(14):1449-1456. Epub 2016 Sep 2 doi: 10.1212/WNL.0000000000003173. PMID: 27590293

Diagnosis

Van Steenhoven RW, de Vries JM, Bruijstens AL, Paunovic M, Nagtzaam MM, Franken SC, Bastiaansen AE, De Bruijn MA, Van Sonderen A, Schreurs MWJ, Gardeniers M, Verdijk RM, Balvers RK, Sillevis Smitt PA, Neuteboom RF, Titulaer MJ
Neurol Neuroimmunol Neuroinflamm 2023 Nov;10(6) Epub 2023 Aug 15 doi: 10.1212/NXI.0000000000200148. PMID: 37582614Free PMC Article
Comperat L, Pegat A, Honnorat J, Joubert B
Curr Opin Neurol 2022 Oct 1;35(5):597-603. Epub 2022 Aug 19 doi: 10.1097/WCO.0000000000001104. PMID: 35989569
Lee WJ, Lee HS, Kim DY, Lee HS, Moon J, Park KI, Lee SK, Chu K, Lee ST
Brain 2022 Oct 21;145(10):3509-3521. doi: 10.1093/brain/awac166. PMID: 35512357
Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596Free PMC Article
van Sonderen A, Thijs RD, Coenders EC, Jiskoot LC, Sanchez E, de Bruijn MA, van Coevorden-Hameete MH, Wirtz PW, Schreurs MW, Sillevis Smitt PA, Titulaer MJ
Neurology 2016 Oct 4;87(14):1449-1456. Epub 2016 Sep 2 doi: 10.1212/WNL.0000000000003173. PMID: 27590293

Therapy

Lee WJ, Lee HS, Kim DY, Lee HS, Moon J, Park KI, Lee SK, Chu K, Lee ST
Brain 2022 Oct 21;145(10):3509-3521. doi: 10.1093/brain/awac166. PMID: 35512357
Costa Fernandes J, Gama Marques J
CNS Spectr 2022 Apr;27(2):191-198. Epub 2020 Oct 30 doi: 10.1017/S1092852920001960. PMID: 33121548
Yang J, Liu X
Front Immunol 2021;12:790962. Epub 2021 Dec 16 doi: 10.3389/fimmu.2021.790962. PMID: 34975890Free PMC Article
Toledano M, Pittock SJ
Semin Neurol 2015 Jun;35(3):245-58. Epub 2015 Jun 10 doi: 10.1055/s-0035-1552625. PMID: 26060904
Derry CP, Wilkie MD, Al-Shahi Salman R, Davenport RJ
Clin Med (Lond) 2011 Oct;11(5):476-8. doi: 10.7861/clinmedicine.11-5-476. PMID: 22034711Free PMC Article

Prognosis

Lee WJ, Lee HS, Kim DY, Lee HS, Moon J, Park KI, Lee SK, Chu K, Lee ST
Brain 2022 Oct 21;145(10):3509-3521. doi: 10.1093/brain/awac166. PMID: 35512357
Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596Free PMC Article
van Sonderen A, Thijs RD, Coenders EC, Jiskoot LC, Sanchez E, de Bruijn MA, van Coevorden-Hameete MH, Wirtz PW, Schreurs MW, Sillevis Smitt PA, Titulaer MJ
Neurology 2016 Oct 4;87(14):1449-1456. Epub 2016 Sep 2 doi: 10.1212/WNL.0000000000003173. PMID: 27590293
Toledano M, Pittock SJ
Semin Neurol 2015 Jun;35(3):245-58. Epub 2015 Jun 10 doi: 10.1055/s-0035-1552625. PMID: 26060904
Derry CP, Wilkie MD, Al-Shahi Salman R, Davenport RJ
Clin Med (Lond) 2011 Oct;11(5):476-8. doi: 10.7861/clinmedicine.11-5-476. PMID: 22034711Free PMC Article

Clinical prediction guides

Mojžišová H, Krýsl D, Hanzalová J, Dargvainiene J, Wandinger KP, Leypoldt F, Elišák M, Marusič P
Neurol Neuroimmunol Neuroinflamm 2023 Nov;10(6) Epub 2023 Oct 25 doi: 10.1212/NXI.0000000000200170. PMID: 37879962Free PMC Article
Van Steenhoven RW, de Vries JM, Bruijstens AL, Paunovic M, Nagtzaam MM, Franken SC, Bastiaansen AE, De Bruijn MA, Van Sonderen A, Schreurs MWJ, Gardeniers M, Verdijk RM, Balvers RK, Sillevis Smitt PA, Neuteboom RF, Titulaer MJ
Neurol Neuroimmunol Neuroinflamm 2023 Nov;10(6) Epub 2023 Aug 15 doi: 10.1212/NXI.0000000000200148. PMID: 37582614Free PMC Article
Lee WJ, Lee HS, Kim DY, Lee HS, Moon J, Park KI, Lee SK, Chu K, Lee ST
Brain 2022 Oct 21;145(10):3509-3521. doi: 10.1093/brain/awac166. PMID: 35512357
Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596Free PMC Article
Lim JA, Lee ST, Moon J, Jun JS, Kim TJ, Shin YW, Abdullah S, Byun JI, Sunwoo JS, Kim KT, Yang TW, Lee WJ, Moon HJ, Kim DW, Lim BC, Cho YW, Yang TH, Kim HJ, Kim YS, Koo YS, Park B, Jung KH, Kim M, Park KI, Jung KY, Chu K, Lee SK
Ann Neurol 2019 Mar;85(3):352-358. Epub 2019 Feb 10 doi: 10.1002/ana.25421. PMID: 30675918

Recent systematic reviews

Vrillon A, Carle G, Berzero G, Honnorat J, Huberfeld G, Psimaras D, Azuar C
Neurosci Biobehav Rev 2020 Dec;119:128-137. Epub 2020 Oct 3 doi: 10.1016/j.neubiorev.2020.08.015. PMID: 33022299
Quartuccio N, Caobelli F, Evangelista L, Alongi P, Kirienko M, De Biasi V, Cocciolillo F; Young AIMN Working Group
J Neuroimmunol 2015 Jul 15;284:44-8. Epub 2015 May 1 doi: 10.1016/j.jneuroim.2015.05.002. PMID: 26025057
Radja GK, Cavanna AE
J Neuropsychiatry Clin Neurosci 2013 Fall;25(4):264-71. doi: 10.1176/appi.neuropsych.13020022. PMID: 24247853

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