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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 Meningitis and Encephalitis.
Richie MB
Neurol Clin 2022 Feb;40(1):93-112. doi: 10.1016/j.ncl.2021.08.007. PMID: 34798977
Neoplastic Meningitis and Paraneoplastic Syndromes.
Kanekar S, Zacharia T, Agarwal A
Radiol Clin North Am 2021 May;59(3):409-423. doi: 10.1016/j.rcl.2021.01.007. PMID: 33926686
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 epilepsy.
Serafini A, Lukas RV, VanHaerents S, Warnke P, Tao JX, Rose S, Wu S
Epilepsy Behav 2016 Aug;61:51-58. Epub 2016 Jun 12 doi: 10.1016/j.yebeh.2016.04.046. PMID: 27304613
Update on neurological paraneoplastic syndromes.
Höftberger R, Rosenfeld MR, Dalmau J
Curr Opin Oncol 2015 Nov;27(6):489-95. doi: 10.1097/CCO.0000000000000222. PMID: 26335665Free PMC Article

Diagnosis

Limbic Encephalitis.
Schattner A, Dubin I, Uliel L
Am J Med 2024 Feb;137(2):e31-e32. Epub 2023 Oct 22 doi: 10.1016/j.amjmed.2023.10.010. PMID: 37875218
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
Limbic encephalitis.
Mahawish K, Teinert L, Cavanagh K, Brennan J
BMJ Case Rep 2014 Jun 2;2014 doi: 10.1136/bcr-2014-204591. PMID: 24891487Free PMC Article
Paraneoplastic limbic encephalitis.
Foster AR, Caplan JP
Psychosomatics 2009 Mar-Apr;50(2):108-13. doi: 10.1176/appi.psy.50.2.108. PMID: 19377018

Therapy

Anti-LGI1 Autoimmune Epilepsy.
Burkett BJ, Johnson DR, Hunt CH, Messina SA, Broski SM
Clin Nucl Med 2023 Nov 1;48(11):956-957. Epub 2023 Sep 7 doi: 10.1097/RLU.0000000000004848. PMID: 37703448
Confused about Confusion.
Spanjaart AM, van der Valk FM, van Rooijen G, Brouwer MC, Kersten MJ
N Engl J Med 2022 Jan 6;386(1):80-87. doi: 10.1056/NEJMcps2114818. PMID: 34986289
Application of Plasma Exchange in Steroid-Responsive Encephalopathy.
Jiang Y, Tian X, Gu Y, Li F, Wang X
Front Immunol 2019;10:324. Epub 2019 Feb 27 doi: 10.3389/fimmu.2019.00324. PMID: 30873174Free PMC Article
Update on neurological paraneoplastic syndromes.
Höftberger R, Rosenfeld MR, Dalmau J
Curr Opin Oncol 2015 Nov;27(6):489-95. doi: 10.1097/CCO.0000000000000222. PMID: 26335665Free PMC Article
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

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
Paraneoplastic epilepsy.
Serafini A, Lukas RV, VanHaerents S, Warnke P, Tao JX, Rose S, Wu S
Epilepsy Behav 2016 Aug;61:51-58. Epub 2016 Jun 12 doi: 10.1016/j.yebeh.2016.04.046. PMID: 27304613
What should you know about limbic encephalitis?
Machado S, Pinto AN, Irani SR
Arq Neuropsiquiatr 2012 Oct;70(10):817-22. doi: 10.1590/s0004-282x2012001000012. PMID: 23060110

Clinical prediction guides

Ageing-Related Neurodegeneration and Cognitive Decline.
Alafuzoff I, Libard S
Int J Mol Sci 2024 Apr 5;25(7) doi: 10.3390/ijms25074065. PMID: 38612875Free 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

Paraneoplastic Syndromes in Neuroendocrine Prostate Cancer: A Systematic Review.
Abufaraj M, Ramadan R, Alkhatib A
Curr Oncol 2024 Mar 21;31(3):1618-1632. doi: 10.3390/curroncol31030123. PMID: 38534956Free PMC Article
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
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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