We present a rare case of hemorrhagic leukoencephalitis (HLE), highlighting its diagnostic complexity and rapid progression.

Hemorrhagic leukoencephalitis (HLE) is a rare, often fatal form of acute encephalitis characterized by inflammation, demyelination, and hemorrhage within the white matter. It is typically triggered by viral infections or vaccinations, leading to an exaggerated autoimmune response. Patients present with rapidly progressive neurological decline. MRI shows multifocal hemorrhagic lesions with edema, and cerebrospinal fluid (CSF) analysis often reveals elevated white blood cell count and protein levels. Despite aggressive treatment, the prognosis remains poor.

A 68-year-old male with hypertension and hyperlipidemia presented with a one-week history of transient headaches and worsening visual disturbances, described as a black spot with a halo. An ophthalmologic evaluation was unremarkable. MRI revealed multifocal hemorrhagic lesions in the cerebellum, occipital lobes, and right frontal lobe with surrounding edema. Initial differential diagnoses included posterior reversible encephalopathy syndrome (PRES), metastases, vasculitis, and demyelinating disorders.

Over 48-96 hours, the patient developed a fever and rapid neurological decline. Lumbar puncture revealed elevated white blood cell count (195/µL) and protein (99 mg/dL), with other CSF and bloodwork within normal limits. Repeat MRIs showed progression of hemorrhagic lesions involving both infratentorial and supratentorial regions. Despite aggressive treatment, including high-dose intravenous steroids and ventriculostomy for hydrocephalus, the patient’s condition deteriorated, leading to death.

Diagnosis of hemorrhagic leukoencephalitis was made.  

Hemorrhagic leukoencephalitis is a devastating, rapidly progressing condition often associated with viral triggers. This case highlights its hallmark features and the absence of a definitive cause, emphasizing the need for further research into its etiology and early diagnosis for improved outcomes.