title: Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection.
abstract: A 16-year-old girl presented signs of a common cold in combination with a hemolytic crisis. Within 3 days, she developed reduced consciousness and hemiparesis subsequently followed by coma. CT and MRI scans revealed evidence for raised intracranial pressure and an extensive inflammatory process extending from the brain stem up to the thalamus. The patient died within 3 weeks after onset of first symptoms of intracranial pressure despite maximum intensive care. Neuropathological examination revealed disseminated necrotic lesions and perivascular hemorrhages characteristic for acute hemorrhagic leukoencephalitis (Hurst's disease), mainly of the brain stem, diencephalon and cerebellum. Serological results, in situ hybridization and PCR analysis demonstrated an acute Epstein-Barr virus (EBV) infection of the central nervous system. To our knowledge, this is the first reported case of Hurst's disease linked to EBV.
authors.affiliation: Department of Neuropathology, Institute for Pathology, University Hospital Freiburg, Breisacherstr. 64, 79106, Freiburg, Germany, hofer@nz.ukl.uni-freiburg.de.
authors.forename: Markus
authors.initials: M
authors.lastname: Hofer
authors.forename: Achim
authors.initials: A
authors.lastname: Weber
authors.forename: Karsten
authors.initials: K
authors.lastname: Haffner
authors.forename: Ansgar
authors.initials: A
authors.lastname: Berlis
authors.forename: Karin
authors.initials: K
authors.lastname: Klingel
authors.forename: Marcus
authors.initials: M
authors.lastname: Krüger
authors.forename: Reinhard
authors.initials: R
authors.lastname: Kandolf
authors.forename: Benedikt
authors.initials: B
authors.lastname: Volk
mesh_terms: D000293:Adolescent; D015703:Antigens, CD / Q000276:immunology; D015214:Antigens, Differentiation, Myelomonocytic / Q000276:immunology; D015152:Blotting, Northern / Q000379:methods; D001921:Brain / Q000378:metabolism / Q000473:pathology / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D017403:In Situ Hybridization / Q000379:methods; D004684:Leukoencephalitis, Acute Hemorrhagic / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D012333:RNA, Messenger / Q000096:biosynthesis; D012367:RNA, Viral / Q000378:metabolism; D020133:Reverse Transcriptase Polymerase Chain Reaction / Q000379:methods; D013194:Staining and Labeling / Q000379:methods; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D005904:Glial Fibrillary Acidic Protein; D012333:RNA, Messenger; D012367:RNA, Viral; C067980:CD68 antigen, human; D000097382:CD68 Molecule; C099537:Epstein-Barr virus encoded RNA 1