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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

Mimics of Autoimmune Encephalitis: Validation of the 2016 Clinical Autoimmune Encephalitis Criteria.
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
Rituximab treatment for autoimmune limbic encephalitis in an institutional cohort.
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
Recognizing Autoimmune-Mediated Encephalitis in the Differential Diagnosis of Limbic Disorders.
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

Antibody-Negative Autoimmune Encephalitis: A Single-Center Retrospective Analysis.
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
Mimics of Autoimmune Encephalitis: Validation of the 2016 Clinical Autoimmune Encephalitis Criteria.
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
Autoimmune neuromyotonia.
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
Seronegative autoimmune encephalitis: clinical characteristics and factors associated with outcomes.
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
Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up.
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

Mimics of Autoimmune Encephalitis: Validation of the 2016 Clinical Autoimmune Encephalitis Criteria.
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
Autoimmune neuromyotonia.
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
Seronegative autoimmune encephalitis: clinical characteristics and factors associated with outcomes.
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
Autoimmune Epilepsy.
Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596Free PMC Article
Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up.
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

Seronegative autoimmune encephalitis: clinical characteristics and factors associated with outcomes.
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
A scoping review on paraneoplastic autoimmune limbic encephalitis (PALE) psychiatric manifestations.
Costa Fernandes J, Gama Marques J
CNS Spectr 2022 Apr;27(2):191-198. Epub 2020 Oct 30 doi: 10.1017/S1092852920001960. PMID: 33121548
Immunotherapy for Refractory Autoimmune Encephalitis.
Yang J, Liu X
Front Immunol 2021;12:790962. Epub 2021 Dec 16 doi: 10.3389/fimmu.2021.790962. PMID: 34975890Free PMC Article
Autoimmune Epilepsy.
Toledano M, Pittock SJ
Semin Neurol 2015 Jun;35(3):245-58. Epub 2015 Jun 10 doi: 10.1055/s-0035-1552625. PMID: 26060904
Autoimmune limbic encephalitis.
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

Seronegative autoimmune encephalitis: clinical characteristics and factors associated with outcomes.
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
Autoimmune Epilepsy.
Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596Free PMC Article
Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up.
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
Autoimmune Epilepsy.
Toledano M, Pittock SJ
Semin Neurol 2015 Jun;35(3):245-58. Epub 2015 Jun 10 doi: 10.1055/s-0035-1552625. PMID: 26060904
Autoimmune limbic encephalitis.
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

Antibody-Negative Autoimmune Encephalitis: A Single-Center Retrospective Analysis.
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
Mimics of Autoimmune Encephalitis: Validation of the 2016 Clinical Autoimmune Encephalitis Criteria.
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
Seronegative autoimmune encephalitis: clinical characteristics and factors associated with outcomes.
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
Autoimmune Epilepsy.
Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596Free PMC Article
Development of the clinical assessment scale in autoimmune encephalitis.
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

Psychiatric symptoms in anti glutamic acid decarboxylase associated limbic encephalitis in adults: a systematic review.
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
The role of PET/CT in the evaluation of patients affected by limbic encephalitis: A systematic review of the literature.
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
Treatment of VGKC complex antibody-associated limbic encephalitis: a systematic review.
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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