scholarly journals Case Report: Epstein–Barr Virus Encephalitis Complicated With Brain Stem Hemorrhage in an Immune-Competent Adult

2021 ◽  
Vol 12 ◽  
Author(s):  
Lingtong Huang ◽  
Xuan Zhang ◽  
Xueling Fang

Encephalitis caused by Epstein-Barr virus infection is uncommon, but most patients have a good outcome after symptomatic treatment. The infiltration of mononuclear cells in blood vessels and necrosis resulting from the immune response to Epstein-Barr virus infection in a very small number of patients seem to be the main cause of death. We describe a fatal case of Epstein-Barr virus encephalitis diagnosed by next-generation sequencing in an immune-competent adult but progressed to brainstem hemorrhage.

Blood ◽  
2004 ◽  
Vol 103 (4) ◽  
pp. 1244-1252 ◽  
Author(s):  
Harutaka Katano ◽  
Mir A. Ali ◽  
Andriani C. Patera ◽  
Marta Catalfamo ◽  
Elaine S. Jaffe ◽  
...  

Abstract Chronic active Epstein-Barr virus infection (CAEBV) is a rare disease in which previously healthy persons develop severe, life-threatening illness. Mutations in the perforin gene have been found in familial hemophagocytic lymphohistiocytosis, which shares some features with CAEBV. We studied a patient who died at age 18, 10 years after the onset of CAEBV. The patient had high titers of antibodies to EBV, EBV RNA in lymph nodes, T-cell lymphoproliferative disease, and hemophagocytic lymphohistiocytosis. DNA sequencing showed novel mutations in both alleles of the perforin gene that resulted in amino acid changes in the protein. The quantity of the native form of perforin from the patient's stimulated peripheral blood mononuclear cells (PBMCs) was extremely low and immunoblotting showed accumulation of an uncleaved precursor form of perforin. Stimulated PBMCs from the patient were defective for Fas-independent cytotoxicity. These data imply that mutations in this patient resulted in reduced perforin-mediated cytotoxicity by his lymphocytes. This is the first case in which perforin mutations have been shown to result in accumulation of the uncleaved, immature form of perforin. Mutations in the perforin gene are associated with some cases of CAEBV with hemophagocytic lymphohistiocytosis.


1980 ◽  
Vol 56 (661) ◽  
pp. 794-795 ◽  
Author(s):  
M. H. Davies ◽  
P. Morgan-Capner ◽  
B. Portmann ◽  
S. P. Wilkinson ◽  
R. Williams

1986 ◽  
Vol 39 (12) ◽  
pp. 1317-1322 ◽  
Author(s):  
P Morgan-Capner ◽  
J A Morris ◽  
M B McIllmurray ◽  
J A Thomas ◽  
D H Crawford ◽  
...  

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