Acute necrotizing hemorrhagic leukoencephalitis

Anthony T Reder MD (Dr. Reder of the University of Chicago served on advisory boards and as a consultant for Bayer, Biogen Idec, Caremark Rx, Genentech, Genzyme, Novartis, Questcor/Malinkrodt, Serono, and Teva-Marion.)
Originally released January 24, 1996; last updated August 14, 2014; expires August 14, 2017

This article includes discussion of acute necrotizing hemorrhagic leukoencephalitis, acute hemorrhagic encephalitis, acute hemorrhagic leukoencephalitis, acute necrotizing hemorrhagic encephalomyelitis, and cute necrotizing hemorrhagic encephalopathy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Acute hemorrhagic leukoencephalitis of Weston Hurst is at the extreme end of the spectrum of demyelinating diseases. It typically follows a viral upper respiratory infection and evolves rapidly to coma and death. "Ball and ring" hemorrhages appear in the centrum semiovale of the brain, associated with mononuclear and neutrophil infiltrates, surrounded by demyelination spreading out from fibrin-filled venules. In this article, the author addresses these issues and suggests that IL-17, a cytokine that attracts neutrophils, may be important in pathogenesis.

Key points

 

• Acute necrotizing hemorrhagic leukoencephalitis is a devastating disorder that often follows a virus or mycoplasma infection.

 

• Lesions are hemorrhagic, with fibrinoid necrosis and polymorphonuclear neutrophil infiltration that is associated with demyelination.

 

• The devastating immune response is from excess sensitization to 1 or more unknown antigens.

 

• Therapy is difficult, and most of those affected will die. Several therapies are suggested to deactivate the immune response.

Historical note and terminology

E. Weston Hurst was the first to clearly define acute necrotizing hemorrhagic leukoencephalitis and to suggest that it was a demyelinating disease (Hurst 1941). He described the abrupt onset of neurologic symptoms following a nonspecific upper respiratory infection in 3 patients: a 43-year-old housewife, a 33-year-old munitions worker, and a 37-year-old accountant. Death occurred in several days, and autopsies of 2 of these patients showed identical hemorrhagic lesions in the white matter. Other authors, such as Gayet, Leyden, Strümpell, Bückler, Leichtenstern, Brie, Oppenheim, de Faro, Baker, and Levy, described similar encephalitic conditions with petechial hemorrhage and inflammation, as early as 1875 (Lander 1958). More cases were detailed in the 10 years after the 1941 report (Adams et al 1949; Crawford 1954).

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