An autopsy case of chronic active Epstein–Barr virus infection (CAEBV): Distribution of central nervous system (CNS) lesions

Kobayashi, Zen; Tsuchiya, Kuniaki; Takahashi, Makoto; Yokota, Osamu; Sasaki, Atsushi; Bhunchet, Ekapot; Arai, Tetsuaki; Akiyama, Haruhiko; Kamoshita, Masaharu; Kotera, Minoru; Mizusawa, Hidehiro

doi:10.1016/j.jns.2008.07.035

Cited by 17 publications

(8 citation statements)

References 12 publications

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“…This case does not fulfill the criteria for CAEBV because of the absence of hepatosplenomegaly and significant elevations in anti-EBV VCA-IgG and EA-IgG antibodies. 2 However, Kobayashi et al 10 reported an autopsy case of CAEBV with CNS lesions showing perivascular CD3+ and EBER1+ lymphocyte infiltration, which resembles the CNS pathology of our case. These cases suggest the disease spectrum of EBV vasculitic pathology in the CNS; however, further investigations are needed.…”

Section: Discussionsupporting

confidence: 60%

Biopsy‐proven case of Epstein–Barr virus (EBV)‐associated vasculitis of the central nervous system

et al. 2016

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A 75-year-old woman was admitted to our hospital with rapidly deteriorating consciousness disturbance. She had a 7-year history of rheumatoid arthritis (RA), which had been treated with methotrexate (MTX) and prednisolone. Brain T2-weighted MRI showed diffuse high-intensity lesions in the cerebral subcortical and deep white matter, bilateral basal ganglia and thalamus. A cerebrospinal fluid examination revealed elevated protein levels and positive Epstein-Barr virus (EBV) DNA. Human immunodeficiency virus was negative. Brain biopsy showed perivascular lymphocytic infiltration in the parenchyma and meninx with EBV-encoded small RNA (EBER). Since this case did not fulfill the criteria for chronic active EBV infection (CAEBV), she was diagnosed with Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system. High-dose methylprednisolone, acyclovir, ganciclovir and foscarnet were not effective. Although EBV is a causative agent of infectious mononucleosis (IM), lymphomas and nasopharyngeal carcinomas, vasculitic pathology of the central nervous system with EBV reactivation in the elderly is rare. Immunosuppressive drugs such as steroids and MTX are widely used to treat autoimmune disorders, but may exacerbate the reactivation of EBV. This is the first case of biopsy-proven EBV-positive/HIV-negative vasculitis during the treatment of RA with MTX and steroids. This case indicates that EBV-associated vasculitis needs to be considered as a differential diagnosis of CNS vasculitis.

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Section: Discussionsupporting

confidence: 60%

Biopsy‐proven case of Epstein–Barr virus (EBV)‐associated vasculitis of the central nervous system

et al. 2016

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“…2, and whose onsets were clear after 20 years of age. Eighteen reported patients were selected as shown in Table 2 [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21].…”

Section: Analysis Of Reported Casesmentioning

confidence: 99%

Clinical features of adult-onset chronic active Epstein–Barr virus infection: a retrospective analysis

et al. 2011

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We performed a retrospective analysis of patients with adult-onset chronic active Epstein-Barr virus infection (CAEBV). First, we analyzed five patients (aged 28-72) diagnosed at our hospitals with EBV-infected clonally proliferating T cells. Four patients were administered cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP) chemotherapy, but no remarkable decrease of viral load was observed in three of the patients. The other patient died 19 days after initiation of CHOP treatment due to disease progression. Addition of high-dose cytarabine to the regimens of two of the patients was discontinued shortly after administration, due to the development of grade 4 pericardial effusion. Together, these regimens may be insufficient for treating adult-onset CAEBV. We next reviewed 23 adult-onset CAEBV patients, adding 18 previously reported patients to the five patients described in the present study. T cells were frequently infected (87%), whereas NK- and T-cell types are known to be almost equally prevalent in childhood-onset cases. The time duration from the onset of disease to initiation of treatment averaged 20 months. Reports showed that 12 patients died; seven patients died at an average of 8 months after initiation of treatment. Patients' disease courses seemed to be rapidly progressive and more aggressive than those of childhood-onset cases. More cases must be studied to clarify clinical features and establish an optimal treatment strategy.

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“…Two previous reports have demonstrated infiltration of EBV-infected T cells into various tissues, including the central nervous system, in patients with CAEBV by becoming vasculitic (15,16). The authors considered that infiltrated EBV-infected T cells directly cause neurological symptoms.…”

Section: Discussionmentioning

confidence: 99%

Immune-mediated Neuropathy with Epstein-Barr virus-positive T-cell Lymphoproliferative Disease

et al. 2015

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A 47-year-old man with Epstein-Barr virus (EBV)-positive T/NK-cell lymphoproliferative disease (EBV-T/NK-LPD) developed acute-onset weakness. A nerve conduction study showed a conduction block in both the proximal and most distal segments. Although the patient's neuropathy transiently responded to intravenous immunoglobulin, it was progressive for at least 25 days until the start of prednisolone (PSL) administration, after which it remarkably improved. The neuropathy further improved after allogeneic bone marrow transplantation (BMT). The present patient's clinical course is not consistent with that of typical GuillainBarré syndrome. This case suggests that EBV-T/NK-LPD can cause progressive immune-mediated neuropathy as a result of chronic EBV antigen presentation and can be treated with PSL and BMT.

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An autopsy case of chronic active Epstein–Barr virus infection (CAEBV): Distribution of central nervous system (CNS) lesions

Cited by 17 publications

References 12 publications

Biopsy‐proven case of Epstein–Barr virus (EBV)‐associated vasculitis of the central nervous system

Biopsy‐proven case of Epstein–Barr virus (EBV)‐associated vasculitis of the central nervous system

Clinical features of adult-onset chronic active Epstein–Barr virus infection: a retrospective analysis

Immune-mediated Neuropathy with Epstein-Barr virus-positive T-cell Lymphoproliferative Disease

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