Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection.
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.
Hofer
Markus
M
Department of Neuropathology, Institute for Pathology, University Hospital Freiburg, Breisacherstr. 64, 79106, Freiburg, Germany, hofer@nz.ukl.uni-freiburg.de.
Weber
Achim
A
Haffner
Karsten
K
Berlis
Ansgar
A
Klingel
Karin
K
Krüger
Marcus
M
Kandolf
Reinhard
R
Volk
Benedikt
B
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
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