Acute Hemorrhagic Leukoencephalopathy: Pathological Features and Cerebrospinal Fluid Cytokine Profiles.

BACKGROUND: Acute hemorrhagic leukoencephalopathy is a rare encephalopathy of unknown etiology, causing fulminant, hemorrhagic central nervous system demyelination with high mortality. It is unclear whether acute hemorrhagic leukoencephalopathy is an entirely distinct entity from acute disseminated encephalomyelitis. PATIENTS AND METHODS: We report two patients with rapidly progressive neurological illness resulting in raised intracranial pressure and coma, with biopsy-proven acute hemorrhagic leukoencephalopathy (perivascular hemorrhages and demyelination, predominantly neutrophil infiltrates). RESULTS: Acute cerebrospinal fluid showed pronounced T cell-associated cytokine elevation (interleukins 6, 8, and 17A) and CCL2 or CCL3, higher than in patients with acute disseminated encephalomyelitis, but no B cell-associated cytokine elevation. CONCLUSION: Improved understanding of the immune process may provide rationale for use of anticytokine biologic agents.

Acute hemorrhagic leukoencephalitis: report of three cases and review of the literature.

Attention is directed to the diagnosis of acute hemorrhagic leukoencephalitis and the possibility of its clinical recognition is discussed. Three case histories, two diagnosed at autopsy and one clinically, are presented. The latter was treated with very generous doses of steroids and recovered spectacularly. On omission of steroids, a mild, temporary clinical recurrence occurred. Clinical, clinico-pathologic, and pathologic aspects of the disease are discussed, and etiologic factors mentioned. Some thoughts on the relationship of this disease to experimental allergic encephalomyelitis are mentioned. Differential diagnosis is considered and the use of biopsy for confirmation of diagnosis especially from herpes encephalitis considered. A plea is made for the trial of treatment with large doses of steroids.