Fulminant Acute Hemorrhagic Leukoencephalitis (Hurst Disease) Mimicking Hemorrhagic Septic Emboli

Abstract

Acute hemorrhagic leukoencephalitis (AHLE), or Hurst disease, is a rare fulminant inflammatory demyelinating disorder characterized by hemorrhagic white-matter injury, marked cerebral edema, and rapid neurological deterioration. A previously healthy 27-year-old man presented with altered mental status after a 7-day febrile illness and rapidly developed stroke-like deficits, quadriplegia, coma, and hypoxemia. Laboratory evaluation showed marked peripheral leukocytosis, elevated D-dimer, prolonged activated partial thromboplastin time, and inflammatory cerebrospinal fluid abnormalities with pleocytosis, mildly elevated protein, and oligoclonal-band positivity. Blood cultures were sterile and sputum examination for acid-fast bacilli was negative. Non-contrast CT demonstrated numerous bilateral asymmetric hemorrhagic parenchymal lesions involving the supratentorial and infratentorial compartments, with extensive surrounding hypoattenuating edema and diffuse cerebral swelling; the basal ganglia were relatively spared. Septic embolic disease with hemorrhagic transformation was initially considered. Brain biopsy demonstrated vasculocentric hemorrhagic inflammatory injury with perivascular hemorrhage, fibrinoid vascular necrosis, neutrophil-predominant inflammation, and acute perivenular demyelination, establishing AHLE. Despite intensive supportive care, the patient died on the third hospital day. AHLE should be considered in rapidly progressive febrile encephalopathy when multifocal non-territorial hemorrhagic lesions are accompanied by disproportionate edema.