title: Biopsy-proven case of Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system.
abstract: 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.
authors.affiliation: Division of Neurology, First Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Kohei
authors.initials: K
authors.lastname: Kano
authors.affiliation: Division of Neurology, First Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Katayama
authors.affiliation: Division of Neurology, First Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Shiori
authors.initials: S
authors.lastname: Takeguchi
authors.affiliation: Division of Neurology, First Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Asuka
authors.initials: A
authors.lastname: Asanome
authors.affiliation: Division of Neurology, First Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Kae
authors.initials: K
authors.lastname: Takahashi
authors.affiliation: Division of Neurology, First Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Saito
authors.affiliation: Division of Neurology, First Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Sawada
authors.affiliation: Department of Neurosurgery, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Masato
authors.initials: M
authors.lastname: Saito
authors.affiliation: Department of Neurosurgery, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Ryogo
authors.initials: R
authors.lastname: Anei
authors.affiliation: Department of Neurosurgery, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Kyousuke
authors.initials: K
authors.lastname: Kamada
authors.affiliation: Department of Clinical Pathology, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Miyokawa
authors.affiliation: Department of Translational Pathology, Hokkaido University School of Medicine, Sapporo, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nishihara
authors.affiliation: Division of Neurology, First Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Hasebe
mesh_terms: D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001172:Arthritis, Rheumatoid / Q000150:complications / Q000188:drug therapy; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology* / Q000821:virology*; D002490:Central Nervous System / Q000473:pathology / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008727:Methotrexate / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D014657:Vasculitis / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000821:virology*
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D011239:Prednisolone; D008727:Methotrexate
keywords: Epstein-Barr virus (EBV); central nervous system; encephalopathy; vasculitis