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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
Updates in the Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes.
Grativvol RS, Cavalcante WCP, Castro LHM, Nitrini R, Simabukuro MM
Curr Oncol Rep 2018 Nov 10;20(11):92. doi: 10.1007/s11912-018-0721-y. PMID: 30415318
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

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
Neuroimmune disorders in COVID-19.
Ariño H, Heartshorne R, Michael BD, Nicholson TR, Vincent A, Pollak TA, Vogrig A
J Neurol 2022 Jun;269(6):2827-2839. Epub 2022 Mar 30 doi: 10.1007/s00415-022-11050-w. PMID: 35353232Free PMC Article
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
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

Limbic encephalitis.
Bien CG
Handb Clin Neurol 2022;187:467-487. doi: 10.1016/B978-0-12-823493-8.00024-9. PMID: 35964988
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
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
Diagnosing autoimmune limbic encephalitis.
Budhram A, Leung A, Nicolle MW, Burneo JG
CMAJ 2019 May 13;191(19):E529-E534. doi: 10.1503/cmaj.181548. PMID: 31085562Free PMC Article

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

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
Paraneoplastic neurological syndromes.
Graus F, Dalmau J
Curr Opin Neurol 2012 Dec;25(6):795-801. doi: 10.1097/WCO.0b013e328359da15. PMID: 23041955Free PMC Article

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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