Summary. Introduction. Acute hemorrhagic leukoencephalitis (AHL), or Hurst disease is a rare, usually fatal, disease, probably due
to an autoimmune cross reaction against myelin antigens present in the central nervous system, and which forms a spectrum of postinfeccious
demyelinating diseases with acute disseminated encephalomyelitis. Case report. The patient was a 21 year old female who
presented with an acute encephalopathy and generalized seizures following a 15 day febrile syndrome attributed to amygdalitis; a
laboratory work-up, including CSF, was non-diagnostic, and a brain CT scan revealed diffuse cerebral edema. After 12 days the
patient died from nosocomial pneumonia and multi-organ failure; neuropathological examination of the brain confirmed the
diagnosis of Hurst acute hemorrhagic leukoencephalitis, with a weak perilesional inflammatory reaction, unlike the usual picture
in AHL. Discussion. AHL should be a part of the differential diagnosis of acute encephalopathic diseases, particularly if preceded
by systemic infections. The atypical laboratory findings, and the impossibilty of performing a brain MRI were obstacles to the
diagnosis in this case. The relative paucity of the perivascular infiltrate is an atypical finding, and could be due to apoptotic clearance
of the inflammatory cells, as has been described in other autoimmune demyelinating diseases
