Extract

To the Editor:

Acute hemorrhagic leukoencephalitis (AHLE), also known as Weston Hurst syndrome,1 is a rare fulminant encephalopathy often preceded by viral infection, including COVID-19 or vaccination.2,3 Due to high mortality, the diagnosis is typically made empirically based on clinical presentation for early intervention or at the time of autopsy. Rarely do patients undergo brain biopsy for diagnostic purposes. In this report, we present an AHLE case with 2 consecutive biopsies that illustrate the histologic evolution of the disease.

A 31-year-old previously healthy woman presented with 1-day of progressive fever, confusion, headache, blurry vision, and left-sided weakness. She reportedly had upper respiratory symptoms a week prior. Neurological examination revealed inattention, left hemineglect, left homonymous hemianopsia, left hemiparesis, and left hyperreflexia with Babinski sign present. No meningismus was seen. Brain MRI revealed a rapidly evolving large right parieto-occipital lesion (Figure 1). Differential diagnosis based on imaging included tumefactive demyelinating lesion, acute demyelinating encephalomyelitis (ADEM), hemophagocytic lymphohistiocytosis, or central nervous system (CNS) infection. Lymphoma or glial neoplasm was less likely given the acute presentation. Vasculitis was also less likely given the absence of changes in a vascular distribution. Laboratory evaluation (Table 1) was notable for positive Rhinovirus species PCR, sputum culture growing Haemophilus influenzae, and elevated serum interleukin-2 receptor. CT chest, abdomen, and pelvis were unremarkable.

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