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Limbic encephalitis

MedGen UID:
137895
•Concept ID:
C0338430
•
Disease or Syndrome
Synonyms: Encephalitides, Paraneoplastic Limbic; Encephalitis, Limbic; Encephalitis, Paraneoplastic Limbic; Limbic Encephalitis; Limbic Encephalitis, Paraneoplastic; Paraneoplastic Limbic Encephalitis
SNOMED CT: Paraneoplastic limbic encephalitis (445014002); Limbic encephalopathy (230192003); Limbic encephalitis (230192003)
 
Monarch Initiative: MONDO:0015588

Definition

A rare disorder characterized by degenerative changes in the limbic area of the brain. Causes include infections and autoimmune conditions; it may also manifest as a paraneoplastic syndrome, most often caused by small cell lung carcinoma. Signs and symptoms include behavioral changes, hallucinations and dementia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLimbic encephalitis
Follow this link to review classifications for Limbic encephalitis in Orphanet.

Professional guidelines

PubMed

Autoimmune Encephalitis: Current Knowledge on Subtypes, Disease Mechanisms and Treatment.
Nissen MS, Ryding M, Meyer M, Blaabjerg M
CNS Neurol Disord Drug Targets 2020;19(8):584-598. doi: 10.2174/1871527319666200708133103. PMID: 32640967
Paraneoplastic syndromes: an approach to diagnosis and treatment.
Pelosof LC, Gerber DE
Mayo Clin Proc 2010 Sep;85(9):838-54. doi: 10.4065/mcp.2010.0099. PMID: 20810794Free PMC Article
Hashimoto's encephalopathy : epidemiology, pathogenesis and management.
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Recent clinical studies

Etiology

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
Autoimmune Meningitis and Encephalitis.
Richie MB
Neurol Clin 2022 Feb;40(1):93-112. doi: 10.1016/j.ncl.2021.08.007. PMID: 34798977
Autoimmune Encephalitis: Current Knowledge on Subtypes, Disease Mechanisms and Treatment.
Nissen MS, Ryding M, Meyer M, Blaabjerg M
CNS Neurol Disord Drug Targets 2020;19(8):584-598. doi: 10.2174/1871527319666200708133103. PMID: 32640967
Understanding and managing acute encephalitis.
Kumar R
F1000Res 2020;9 Epub 2020 Jan 29 doi: 10.12688/f1000research.20634.1. PMID: 32047620Free PMC Article
Alzheimer's Disease Including Focal Presentations.
Villain N, Dubois B
Semin Neurol 2019 Apr;39(2):213-226. Epub 2019 Mar 29 doi: 10.1055/s-0039-1681041. PMID: 30925614

Diagnosis

Autoimmune-mediated encephalitis and mimics: A neuroimaging review.
Liang C, Chu E, Kuoy E, Soun JE
J Neuroimaging 2023 Jan;33(1):19-34. Epub 2022 Oct 10 doi: 10.1111/jon.13060. PMID: 36217010
Limbic encephalitis.
Bien CG
Handb Clin Neurol 2022;187:467-487. doi: 10.1016/B978-0-12-823493-8.00024-9. PMID: 35964988
Neurological Syndromes Associated with Anti-GAD Antibodies.
Dade M, Berzero G, Izquierdo C, Giry M, Benazra M, Delattre JY, Psimaras D, Alentorn A
Int J Mol Sci 2020 May 24;21(10) doi: 10.3390/ijms21103701. PMID: 32456344Free PMC Article
Autoimmune Encephalitides.
Younger DS
Neurol Clin 2019 May;37(2):359-381. Epub 2019 Mar 16 doi: 10.1016/j.ncl.2019.01.015. PMID: 30952414
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
LGI1 antibody encephalitis: acute treatment comparisons and outcome.
Rodriguez A, Klein CJ, Sechi E, Alden E, Basso MR, Pudumjee S, Pittock SJ, McKeon A, Britton JW, Lopez-Chiriboga AS, Zekeridou A, Zalewski NL, Boeve BF, Day GS, Gadoth A, Burkholder D, Toledano M, Dubey D, Flanagan EP
J Neurol Neurosurg Psychiatry 2022 Mar;93(3):309-315. Epub 2021 Nov 25 doi: 10.1136/jnnp-2021-327302. PMID: 34824144Free PMC Article
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
Hashimoto's encephalopathy : epidemiology, pathogenesis and management.
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Prognosis

Limbic encephalitis.
Bien CG
Handb Clin Neurol 2022;187:467-487. doi: 10.1016/B978-0-12-823493-8.00024-9. PMID: 35964988
Autoimmune Encephalitis: Current Knowledge on Subtypes, Disease Mechanisms and Treatment.
Nissen MS, Ryding M, Meyer M, Blaabjerg M
CNS Neurol Disord Drug Targets 2020;19(8):584-598. doi: 10.2174/1871527319666200708133103. PMID: 32640967
Neurological Syndromes Associated with Anti-GAD Antibodies.
Dade M, Berzero G, Izquierdo C, Giry M, Benazra M, Delattre JY, Psimaras D, Alentorn A
Int J Mol Sci 2020 May 24;21(10) doi: 10.3390/ijms21103701. PMID: 32456344Free PMC Article
Autoimmune Epilepsy.
Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596Free PMC Article
GAD65 neurological autoimmunity.
McKeon A, Tracy JA
Muscle Nerve 2017 Jul;56(1):15-27. Epub 2017 Apr 11 doi: 10.1002/mus.25565. PMID: 28063151

Clinical prediction guides

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
Neurological Syndromes Associated with Anti-GAD Antibodies.
Dade M, Berzero G, Izquierdo C, Giry M, Benazra M, Delattre JY, Psimaras D, Alentorn A
Int J Mol Sci 2020 May 24;21(10) doi: 10.3390/ijms21103701. PMID: 32456344Free PMC Article
Autoimmune Epilepsy.
Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596Free PMC Article
GAD65 neurological autoimmunity.
McKeon A, Tracy JA
Muscle Nerve 2017 Jul;56(1):15-27. Epub 2017 Apr 11 doi: 10.1002/mus.25565. PMID: 28063151

Recent systematic reviews

Autoimmune encephalitis associated with COVID-19: A systematic review.
Nabizadeh F, Balabandian M, Sodeifian F, Rezaei N, Rostami MR, Naser Moghadasi A
Mult Scler Relat Disord 2022 Jun;62:103795. Epub 2022 Apr 6 doi: 10.1016/j.msard.2022.103795. PMID: 35472834Free PMC Article
The Peculiar Clinical Symptoms and Treatment of Limbic Encephalitis Associated with AMPA Receptor Antibody.
Jia Y, Li M, Wang H, Zhang M, Wang Y
Eur Neurol 2021;84(3):206-211. Epub 2021 Apr 15 doi: 10.1159/000515592. PMID: 33857949
A review of EEG in anti-NMDA receptor encephalitis.
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
Immune therapy in autoimmune encephalitis: a systematic review.
Nosadini M, Mohammad SS, Ramanathan S, Brilot F, Dale RC
Expert Rev Neurother 2015;15(12):1391-419. Epub 2015 Nov 24 doi: 10.1586/14737175.2015.1115720. PMID: 26559389
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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