title: Guillain-Barré syndrome and hemophagocytic lymphohistiocytosis in a patient with severe chronic active Epstein-Barr virus infection syndrome.
abstract: Epstein-Barr virus (EBV) infection causes a wide range of neurologic and hematologic manifestations. We report a 72-year-old Japanese male patient with severe chronic active EBV infection syndrome (SCAEBV) who presented with Guillain-Barré syndrome (GBS) and developed hemophagocytic lymphohistiocytosis (HLH) several months after the onset of GBS. He showed acute onset of distal muscle weakness, ophthalmoplegia and bulbar palsy. Results of nerve conduction study revealed acute motor-sensory axonal neuropathy (AMSAN). His serum was positive for anti-LM1 IgG and anti-GM1b IgM. Titers of antibodies to EBV-related antigens indicated chronic reactivated EBV infection. Treatment with IVIg resolved the acute ophthalmoplegia, but there was no notable improvement in the AMSAN and bulbar palsy despite repeated. Finally, he developed refractory HLH resulting in a fatal outcome. In the present patient, it seems that SCAEBV was associated with the development of GBS and fatal HLH via parainfectious autoimmunity and direct infectious immune mechanisms, respectively.
authors.affiliation: Department of Internal Medicine, Anan Kyoei Hospital, Hanoura-cho, Naka-gun, Tokushima, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Takahashi
authors.forename: Makoto
authors.initials: M
authors.lastname: Kunishige
authors.forename: Masayuki
authors.initials: M
authors.lastname: Shinohara
authors.forename: Katsuyuki
authors.initials: K
authors.lastname: Kubo
authors.forename: Hideo
authors.initials: H
authors.lastname: Inoue
authors.forename: Hiide
authors.initials: H
authors.lastname: Yoshino
authors.forename: Atsuko
authors.initials: A
authors.lastname: Asano
authors.forename: Souichi
authors.initials: S
authors.lastname: Honda
authors.forename: Toshio
authors.initials: T
authors.lastname: Matsumoto
authors.forename: Takao
authors.initials: T
authors.lastname: Mitsui
mesh_terms: D000368:Aged; D002908:Chronic Disease; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D017809:Fatal Outcome; D020275:Guillain-Barre Syndrome / Q000175:diagnosis / Q000628:therapy / Q000821:virology*; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000628:therapy / Q000821:virology*; D008297:Male; D013577:Syndrome