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Progressive rubella panencephalitis

MedGen UID:
726815
Concept ID:
C1305924
Disease or Syndrome
Synonyms: progressive rubella panencephalitis; rubella panencephalitis; Rubella panencephalitis
SNOMED CT: Progressive rubella panencephalitis (10082001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019386
Orphanet: ORPHA83616

Definition

A rare chronic encephalitis developing up to several years after congenital rubella virus infection or rubella infection in childhood, characterized by slowly progressive, wide-spread neurological symptoms, like cognitive decline, cerebellar ataxia, spasticity, and seizures, amongst others. Progredient deterioration of the neurological disease eventually leads to the death of the patient. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProgressive rubella panencephalitis

Recent clinical studies

Etiology

Guizzaro A, Volpe E, Lus G, Bravaccio F, Cotrufo R, Paolozzi C
Acta Neurol (Napoli) 1992 Aug-Dec;14(4-6):485-92. PMID: 1293990
Sever JL, South MA, Shaver KA
Rev Infect Dis 1985 Mar-Apr;7 Suppl 1:S164-9. doi: 10.1093/clinids/7.supplement_1.s164. PMID: 4001724
Cooper LZ
Rev Infect Dis 1985 Mar-Apr;7 Suppl 1:S2-10. doi: 10.1093/clinids/7.supplement_1.s2. PMID: 3890105

Diagnosis

Guizzaro A, Volpe E, Lus G, Bravaccio F, Cotrufo R, Paolozzi C
Acta Neurol (Napoli) 1992 Aug-Dec;14(4-6):485-92. PMID: 1293990
Jan JE, Tingle AJ, Donald G, Kettyls M, Buckler WS, Dolman CL
Dev Med Child Neurol 1979 Oct;21(5):648-52. doi: 10.1111/j.1469-8749.1979.tb01680.x. PMID: 92431
Townsend JJ, Baringer JR, Wolinsky JS, Malamud N, Mednick JP, Panitch HS, Scott RA, Oshiro L, Cremer NE
N Engl J Med 1975 May 8;292(19):990-3. doi: 10.1056/NEJM197505082921902. PMID: 1117960

Therapy

Jan JE, Tingle AJ, Donald G, Kettyls M, Buckler WS, Dolman CL
Dev Med Child Neurol 1979 Oct;21(5):648-52. doi: 10.1111/j.1469-8749.1979.tb01680.x. PMID: 92431
Wolinsky JS, Dau PC, Buimovici-Klein E, Mednick J, Berg BO, Lang PB, Cooper LZ
Clin Exp Immunol 1979 Mar;35(3):397-404. PMID: 88287Free PMC Article

Prognosis

Guizzaro A, Volpe E, Lus G, Bravaccio F, Cotrufo R, Paolozzi C
Acta Neurol (Napoli) 1992 Aug-Dec;14(4-6):485-92. PMID: 1293990
Gilden DH
Postgrad Med 1983 Jan;73(1):99-101, 104-8. doi: 10.1080/00325481.1983.11698316. PMID: 6849001
Abe T, Nukada T, Hatanaka H, Tajima M, Hiraiwa M, Ushijima H
Arch Neurol 1983 Feb;40(2):98-100. doi: 10.1001/archneur.1983.04050020060012. PMID: 6824459
Jan JE, Tingle AJ, Donald G, Kettyls M, Buckler WS, Dolman CL
Dev Med Child Neurol 1979 Oct;21(5):648-52. doi: 10.1111/j.1469-8749.1979.tb01680.x. PMID: 92431

Clinical prediction guides

Frey TK
Intervirology 1997;40(2-3):167-75. doi: 10.1159/000150543. PMID: 9450233
Hofman FM, Hinton DR, Baemayr J, Weil M, Merrill JE
Clin Immunol Immunopathol 1991 Mar;58(3):331-42. doi: 10.1016/0090-1229(91)90124-s. PMID: 1900461

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