title: Clinically Mild Encephalitis/Encephalopathy With a Reversible Splenial Lesion Accompanied by Epstein-Barr Virus Hemophagocytic Lymphohistiocytosis: A Case Report and Review of the Literature.
abstract: Central nervous system involvement in hemophagocytic lymphohistiocytosis (HLH) is associated with a poor outcome. For such patients, it is unknown whether more aggressive therapies, such as intrathecal methotrexate or hydrocortisone, are inevitably required. We present a very rare case of 3-year-old Japanese girl who developed mild encephalitis/encephalopathy with a reversible splenial lesion, accompanied by Epstein-Barr virus-associated HLH, and review previous similar reports. Our case and previous reports suggest that mild encephalitis/encephalopathy with a reversible splenial lesion accompanied by Epstein-Barr virus-associated HLH has a relatively good prognosis, even in the absence of intrathecal treatments.
authors.affiliation: Departments of *Emergency and Critical Care Medicine †Hematology and Oncology ‡Neurology, Hyogo Prefectural Kobe Children's Hospital, Kobe §Division of Advanced Medicine for Virus Infections, National Research Institute for Child Health and Development, Tokyo, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yamaguchi
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Ishida
authors.forename: Takehito
authors.initials: T
authors.lastname: Yokoi
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Tanaka
authors.forename: Azusa
authors.initials: A
authors.lastname: Maruyama
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Nagase
authors.forename: Daiichiro
authors.initials: D
authors.lastname: Hasegawa
authors.forename: Ken-Ichi
authors.initials: KI
authors.lastname: Imadome
authors.forename: Hiroki
authors.initials: H
authors.lastname: Takeda
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Kosaka
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Uetani
mesh_terms: D001853:Bone Marrow / Q000473:pathology; D003337:Corpus Callosum / Q000000981:diagnostic imaging / Q000473:pathology*; D016572:Cyclosporine / Q000627:therapeutic use; D003693:Delirium / Q000209:etiology; D003907:Dexamethasone / Q000031:analogs & derivatives / Q000627:therapeutic use; D038524:Diffusion Magnetic Resonance Imaging; D004359:Drug Therapy, Combination; D004660:Encephalitis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology*; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000000981:diagnostic imaging; D005047:Etoposide / Q000627:therapeutic use; D006801:Humans; D007010:Hyponatremia / Q000209:etiology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000821:virology; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D059906:Neuroimaging; D011379:Prognosis; D020551:Pulse Therapy, Drug
chemical_list: D005047:Etoposide; D003907:Dexamethasone; D016572:Cyclosporine; D008775:Methylprednisolone