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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]

Professional guidelines

PubMed

Nissen MS, Ryding M, Meyer M, Blaabjerg M
CNS Neurol Disord Drug Targets 2020;19(8):584-598. doi: 10.2174/1871527319666200708133103. PMID: 32640967
Pelosof LC, Gerber DE
Mayo Clin Proc 2010 Sep;85(9):838-54. doi: 10.4065/mcp.2010.0099. PMID: 20810794Free PMC Article
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

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
Richie MB
Neurol Clin 2022 Feb;40(1):93-112. doi: 10.1016/j.ncl.2021.08.007. PMID: 34798977
Nissen MS, Ryding M, Meyer M, Blaabjerg M
CNS Neurol Disord Drug Targets 2020;19(8):584-598. doi: 10.2174/1871527319666200708133103. PMID: 32640967
Kumar R
F1000Res 2020;9 Epub 2020 Jan 29 doi: 10.12688/f1000research.20634.1. PMID: 32047620Free PMC Article
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

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
Bien CG
Handb Clin Neurol 2022;187:467-487. doi: 10.1016/B978-0-12-823493-8.00024-9. PMID: 35964988
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
Younger DS
Neurol Clin 2019 May;37(2):359-381. Epub 2019 Mar 16 doi: 10.1016/j.ncl.2019.01.015. PMID: 30952414
Toledano M, Pittock SJ
Semin Neurol 2015 Jun;35(3):245-58. Epub 2015 Jun 10 doi: 10.1055/s-0035-1552625. PMID: 26060904

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
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
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
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Prognosis

Bien CG
Handb Clin Neurol 2022;187:467-487. doi: 10.1016/B978-0-12-823493-8.00024-9. PMID: 35964988
Nissen MS, Ryding M, Meyer M, Blaabjerg M
CNS Neurol Disord Drug Targets 2020;19(8):584-598. doi: 10.2174/1871527319666200708133103. PMID: 32640967
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
Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596Free PMC Article
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

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
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
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
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

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
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
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
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
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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