title: An autopsy case of chronic active Epstein-Barr virus infection (CAEBV): distribution of central nervous system (CNS) lesions.
abstract: A 27-year-old Japanese man developed recurrent respiratory and central nervous system (CNS) symptoms, and hemophagocytic syndromes with a clinical course of 6 years. CT demonstrated multiple nodular lesions in the bilateral lungs, and MRI revealed multiple abnormal intensity areas in the brain and spinal cord. Cerebrospinal fluid (CSF) examination disclosed mild pleocytosis and the presence of Epstein-Barr virus (EBV)-DNA detected by polymerase chain reaction (PCR). The patient died of a hemorrhagic shock associated with a hemophagocytic syndrome. A postmortem study revealed massive hemorrhage in the abdominal cavity and iliopsoas muscles, as well as diffuse infiltration of lymphocytes and/or macrophages into the lungs, liver, kidneys, spleen, cardiac muscle, bone marrow, and CNS. The severe involvement was demonstrated in the CNS, especially in the spinal cord and brainstem. The CD3 positive cells of the brainstem were EBV-encoded RNA 1 positive. This is the first autopsy case of chronic active EBV infection (CAEBV) in which severe and extensive CNS involvement was demonstrated.
authors.affiliation: Department of Neurology, Tsuchiura Kyodo Hospital, Ibaraki, 300-0053, Japan. zen@bg7.so-net.ne.jp
authors.forename: Zen
authors.initials: Z
authors.lastname: Kobayashi
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: Makoto
authors.initials: M
authors.lastname: Takahashi
authors.forename: Osamu
authors.initials: O
authors.lastname: Yokota
authors.forename: Atsushi
authors.initials: A
authors.lastname: Sasaki
authors.forename: Ekapot
authors.initials: E
authors.lastname: Bhunchet
authors.forename: Tetsuaki
authors.initials: T
authors.lastname: Arai
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Akiyama
authors.forename: Masaharu
authors.initials: M
authors.lastname: Kamoshita
authors.forename: Minoru
authors.initials: M
authors.lastname: Kotera
authors.forename: Hidehiro
authors.initials: H
authors.lastname: Mizusawa
mesh_terms: D000328:Adult; D002908:Chronic Disease; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009422:Nervous System Diseases / Q000150:complications / Q000473:pathology* / Q000821:virology