title: EBV associated hemophagocytic syndrome accompanied by central pontine myelinolysis.
abstract: The development of central pontine myelinolysis (CPM) has rarely been reported in association with hemophagocytic syndromes (HPS). Here we report a unique case of Epstein-Barr Virus (EBV)-related HPS which was accompanied with CPM. A 72-year-old man who had no significant medical history was admitted to our hospital due to high fever and progressing dysphasia and dysarthria. Physical examination revealed anisocoria of the right pupil, fixed reaction to light, and paralysis of the left vagus nerves. Magnetic resonance imaging revealed low signal intensity on T1-weighted images and high signal intensity T2-weighted images in the patient's central midpontine lesion. Initial work-up showed anemia and thrombocytopenia with elevated levels of serum ferritin, lactate dehydrogenase, and soluble IL-2 receptor. Bone marrow aspiration revealed hemophagocytosis. The EBV genome was detected in the peripheral blood using the polymerase chain reaction method. He was diagnosed as having EBV-related HPS and CPM. Despite intensive treatment with methylpredonisolone, immunoglobulin, and etoposide, he died due to progressive disease and fungal septicemia. The etiology and relation between CPM and HPS are discussed.
authors.affiliation: Department of Internal Medicine, Kagawa Prefectural Central Hospital, Kagawa, 5-4-16, Ban-cho, Takamatsu city 760-8557, Japan.
authors.forename: Eisuke
authors.initials: E
authors.lastname: Uehara
authors.forename: Taizo
authors.initials: T
authors.lastname: Tasaka
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Matsuhashi
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Fujita
authors.forename: Takahiro
authors.initials: T
authors.lastname: Tamura
authors.forename: Minoru
authors.initials: M
authors.lastname: Kuwajima
authors.forename: Masami
authors.initials: M
authors.lastname: Nagai
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Ishida
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D001856:Bone Marrow Examination; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D017809:Fatal Outcome; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D017590:Myelinolysis, Central Pontine / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology