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.
Similar content being viewed by others
References
Adams RD, Cammermeyer J, Denny-Brown D (1949) Acute necrotizing hemorrhagic encephalopathy. J Neuropathol Exp Neurol 8:1–49
Ambler M, Stoll J, Tzamaloukas A, Albala MM (1971) Focal encephalomyelitis in infectious mononucleosis. A report with pathological description. Ann Intern Med 75:579–583
An SF, Groves M, Martinian L, Kuo LT, Scaravilli F (2002) Detection of infectious agents in brain of patients with acute hemorrhagic leukoencephalitis. J Neurovirol 8:439–446
Bray PF, Culp KW, McFarlin DE, Panitch HS, Torkelson RD, Schlight JP (1992) Demyelinating disease after neurologically complicated primary Epstein-Barr virus infection. Neurology 42:278–282
Byers RK (1975) Acute hemorrhagic leukoencephalitis: report of three cases and review of the literature. Pediatrics 56:727–735
Corssmit EP, Leverstein-van Hall MA, Portegies P, Bakker P (1997) Severe neurological complications in association with Epstein-Barr virus infection. J Neurovirol 3:460–464
Fenichel GM (1982) Neurological complications of immunization. Ann Neurol 12:119–128
Ferraro A, Roizin L (1954) Neuropathologic variations in experimental allergic encephalomyelitis, hemorrhagic encephalomyelitis, perivenous encephalomyelitis, diffuse encephalomyelitis, patchy gliosis. J Neuropathol Exp Neurol 13:60–89
Fisher RS, Clark AW, Wolinsky JS, Parhad IM, Moses H, Mardiney MR (1983) Postinfectious leukoencephalitis complicating Mycoplasma pneumoniae infection. Arch Neurol 40:109–113
Fujimoto H, Asaoka K, Imaizumi T, Ayabe M, Shoji H, Kaji M (2003) Epstein-Barr virus infections of the central nervous system. Intern Med 42:33–40
Geerts Y, Dehaene I, Lammens M (1991) Acute hemorrhagic leukoencephalitis. Acta Neurol Belg 91:201–211
Graham DI, Lantos PL (1997) Viral diseases. In: Graham DI, Lantos PL (eds) Greenfield’s neuropathology, 6th edn. Arnold, London, pp 3–64
Gulley ML (2001) Molecular diagnosis of Epstein-Barr virus-related diseases. J Mol Diagn 3:1–10
Hart MN, Earle KM (1975) Haemorrhagic and perivenous encephalitis: a clinical-pathological review of 38 cases. J Neurol Neurosurg Psychiatry 38:585–591
Herrlinger U, Klingel K, Meyermann R, Kandolf R, Kaiserling E, Kortmann RD, Melms A, Skalej M, Dichgans J, Weller M (2000) Central nervous system Hodgkin’s lymphoma without systemic manifestation: case report and review of the literature. Acta Neuropathol 99:709–714
Horwitz CA, Moulds J, Henle W, Henle G, Polesky H, Balfour HH Jr, Schwartz B, Hoff T (1977) Cold agglutinins in infectious mononucleosis and heterophil-antibody-negative mononucleosis-like syndromes. Blood 50:195–202
Hung KL, Liao HT, Tsai ML (2000) Postinfectious encephalomyelitis: etiologic and diagnostic trends. J Child Neurol 15:666–670
Hurst WE (1941) Acute haemorrhagic leucoencephalitis: a previously undefined entity. Med J Aust 11:1–6
Kaluza J, Krygowska-Wajs A, Czapinski P (1998) Acute hemorrhagic encephalitis (Hurst disease) associated with neuroaxonal dystrophy. Folia Neuropathol 36:221–224
Kleinschmidt-DeMasters BK, Gilden DH (2001) The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol 11:440–451
Klingel K, Hohenadl C, Canu A, Albrecht M, Seemann M, Mall G, Kandolf R (1992) Ongoing enterovirus-induced myocarditis is associated with persistent heart muscle infection: quantitative analysis of virus replication, tissue damage, and inflammation. Proc Natl Acad Sci USA 89:314–318
Konig AL, Schabel A, Sugg U, Brand U, Roelcke D (2001) Autoimmune hemolytic anemia caused by IgG lambda-monotypic cold agglutinins of anti-Pr specificity after rubella infection. Transfusion 41:488–492
Poser CM (1969) Disseminad vasculomyelinopathy. A review of the clinical and pathologic reactions of the nervous system in hyperergic diseases. Acta Neurol Scand Suppl 37:3–44
Russell DS (1955) The nosological unity of acute haemorrhagic leucoencephalitis and acute disseminated encephalomyelitis. Brain 78:369–376
Sambrook J, Fritsch EF, Maniatis T (1989) Molecular cloning: a laboratory manual. Cold Spring Harbor Press, Cold Spring Harbor
Terness P, Kirschfink M, Navolan D, Dufter C, Kohl I, Opelz G, Roelcke D (1995) Striking inverse correlation between IgG anti-F(ab′)2 and autoantibody production in patients with cold agglutination. Blood 85:548–551
Todman DH (1983) Encephalitis in infectious mononucleosis. Clin Exp Neurol 19:81–86
Tselis AC, Lisak RP (1995) Acute disseminated encephalomyelitis and isolated central nervous system demyelinative syndromes. Curr Opin Neurol 8:227–229
Acknowledgement
We thank Viviana Sverdlick and Christine el Gaz for their excellent technical assistance.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Hofer, M., Weber, A., Haffner, K. et al. Acute hemorrhagic leukoencephalitis (Hurst’s disease) linked to Epstein-Barr virus infection. Acta Neuropathol 109, 226–230 (2005). https://doi.org/10.1007/s00401-004-0930-3
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00401-004-0930-3