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Encephalitis lethargica

MedGen UID:
4028
Concept ID:
C0014040
Disease or Syndrome
Synonym: Von Economo's disease
SNOMED CT: Encephalitis lethargica (186499007); Von Economo's disease (186499007); von Economo's encephalitis (186499007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019384
Orphanet: ORPHA83600

Definition

A rare brain inflammatory disease characterized by acute or subacute encephalitis with involvement of the midbrain and basal ganglia occurring in children as well as adults. Initial symptoms are pharyngitis and fever, followed by progressive lethargy, sleep disturbances, extrapyramidal symptoms (parkinsonism, chorea, dystonia), neuropsychiatric manifestations (obsessive-compulsive behavior, mutism, catatonia), and ocular features (oculogyric crises). Autoantibodies against human basal ganglia are often positive. Survivors may develop post-encephalitic syndromes, most prominently parkinsonism. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEncephalitis lethargica
Follow this link to review classifications for Encephalitis lethargica in Orphanet.

Recent clinical studies

Etiology

Strong MJ
J Neurochem 2023 Apr;165(2):115-130. Epub 2022 Dec 11 doi: 10.1111/jnc.15736. PMID: 36458986Free PMC Article
Leta V, Urso D, Batzu L, Lau YH, Mathew D, Boura I, Raeder V, Falup-Pecurariu C, van Wamelen D, Ray Chaudhuri K
J Neural Transm (Vienna) 2022 Sep;129(9):1119-1132. Epub 2022 Aug 29 doi: 10.1007/s00702-022-02536-y. PMID: 36036863Free PMC Article
Shannon KM
J Parkinsons Dis 2022;12(s1):S45-S51. doi: 10.3233/JPD-223271. PMID: 35723116Free PMC Article
Berche P
Presse Med 2022 Sep;51(3):104127. Epub 2022 Jun 1 doi: 10.1016/j.lpm.2022.104127. PMID: 35661699
Bond M, Bechter K, Müller N, Tebartz van Elst L, Meier UC
Prog Neuropsychopharmacol Biol Psychiatry 2021 Jul 13;109:110276. Epub 2021 Feb 5 doi: 10.1016/j.pnpbp.2021.110276. PMID: 33549696

Diagnosis

Walusinski O
Rev Neurol (Paris) 2022 Nov;178(9):878-885. Epub 2022 May 12 doi: 10.1016/j.neurol.2022.01.013. PMID: 35568513
Overeem S, van Litsenburg RRL, Reading PJ
Handb Clin Neurol 2021;182:369-385. doi: 10.1016/B978-0-12-819973-2.00025-3. PMID: 34266606
Badenoch J, Searle T, Watson I, Cavanna AE
Neurol Sci 2021 Apr;42(4):1311-1323. Epub 2021 Jan 23 doi: 10.1007/s10072-021-05065-w. PMID: 33486621Free PMC Article
Hoffman LA, Vilensky JA
Brain 2017 Aug 1;140(8):2246-2251. doi: 10.1093/brain/awx177. PMID: 28899018
Lancet 1981 Dec 19-26;2(8260-61):1396-7. PMID: 6118765

Therapy

Smeyne RJ, Eells JB, Chatterjee D, Byrne M, Akula SM, Sriramula S, O'Rourke DP, Schmidt P
Mov Disord 2022 Jul;37(7):1394-1404. Epub 2022 Jun 9 doi: 10.1002/mds.29116. PMID: 35579496Free PMC Article
Hoffman LA, Vilensky JA
Brain 2017 Aug 1;140(8):2246-2251. doi: 10.1093/brain/awx177. PMID: 28899018
Schilder JC, Overmars SS, Marinus J, van Hilten JJ, Koehler PJ
Parkinsonism Relat Disord 2017 Apr;37:27-35. Epub 2017 Jan 22 doi: 10.1016/j.parkreldis.2017.01.010. PMID: 28153525
Hack N, Jicha GA, Abell A, Dean D, Vitek JL, Berger JR
Ann Neurol 2012 Dec;72(6):912-7. doi: 10.1002/ana.23697. PMID: 23280841Free PMC Article
Kapadia F, Grant IS
Intensive Care Med 1990;16(5):338-9. doi: 10.1007/BF01706364. PMID: 2120305

Prognosis

Maurizi CP
Med Hypotheses 2010 May;74(5):798-801. Epub 2010 Jan 8 doi: 10.1016/j.mehy.2009.12.012. PMID: 20060230
Poloni C, Korff CM, Ricotti V, King MD, Perez ER, Mayor-Dubois C, Haenggeli CA, Deonna T
Dev Med Child Neurol 2010 May;52(5):e78-82. Epub 2009 Dec 23 doi: 10.1111/j.1469-8749.2009.03542.x. PMID: 20041934
Ali S, Welch CA, Park LT, Pliakas AM, Wilson A, Nicolson S, Huffman J, Fricchione GL
Cogn Behav Neurol 2008 Mar;21(1):46-51. doi: 10.1097/WNN.0b013e318168464a. PMID: 18327024
Dickman MS
Arch Neurol 2001 Oct;58(10):1696-8. doi: 10.1001/archneur.58.10.1696. PMID: 11594935
Kiley M, Esiri MM
Clin Neuropathol 2001 Jan-Feb;20(1):2-7. PMID: 11220691

Clinical prediction guides

Berche P
Presse Med 2022 Sep;51(3):104127. Epub 2022 Jun 1 doi: 10.1016/j.lpm.2022.104127. PMID: 35661699
Unwin RJ
Kidney Blood Press Res 2021;46(5):639-646. Epub 2021 Oct 18 doi: 10.1159/000519288. PMID: 34662882
Badenoch J, Searle T, Watson I, Cavanna AE
Neurol Sci 2021 Apr;42(4):1311-1323. Epub 2021 Jan 23 doi: 10.1007/s10072-021-05065-w. PMID: 33486621Free PMC Article
Ward CD
Mov Disord 2011 Mar;26(4):599-604. Epub 2011 Feb 10 doi: 10.1002/mds.23545. PMID: 21312282
Schiller F
J Hist Neurosci 2002 Dec;11(4):392-401. doi: 10.1076/jhin.11.4.392.8540. PMID: 12557656

Recent systematic reviews

Badenoch J, Searle T, Watson I, Cavanna AE
Neurol Sci 2021 Apr;42(4):1311-1323. Epub 2021 Jan 23 doi: 10.1007/s10072-021-05065-w. PMID: 33486621Free PMC Article

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