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Hurst’s disease (acute hemorrhagic leukoencephalitis) is a rare fulminant demyelinating disease of the central nervous system. It was singled out as a separate nosological form in the middle of the 20th century. The etiology is not fully understood. According to statistics, the disease most often occurs after a viral or bacterial infection. Patients have an increase in body temperature up to febrile values, headaches, gastrointestinal disorders, cognitive impairments. When making a diagnosis, they are guided by the results of neuroimaging (computed tomography/magnetic resonance imaging), analysis of cerebrospinal fluid, and brain biopsy. Corticosteroids, acyclovir, antibiotics, immunoglobulins, plasmapheresis are used for treatment. The prognosis in patients with acute hemorrhagic leukoencephalitis is poor.

Acute hemorrhagic leukoencephalitis (AHLE), is a rare inflammatory demyelinating disorder, variant of acute disseminated encephalomyelitis. The diagnosis of AHLE remains challenging due to the rarity of the disease and the lack of a reliable biomarker. We report here a case of a 73-year-old male patient with a progressive, low-grade febrile confusional syndrome 20 days after receiving the first dose of BNT162b2 vaccine against SARS-CoV-2. Evidence indicative of the underlying condition by an extensive panel of imaging (brain magnetic resonance imaging, computed tomography and digital subtraction angiography), laboratory (complete blood count, biochemistry, coagulation, tests for autoimmune or infectious disorders, tumor markers, hormonal levels, cerebrospinal fluid analysis) and electrodiagnostic tests were scarce, and mainly non-specific. Sequential neuroimaging revealed the appearance of extensive T2 lesions (signs of gliosis) along with multiple hemorrhagic lesions at various cortical sites. The patient was treated with corticosteroids, discontinued due to severe adverse effects, and subsequently with sessions of plasmapheresis and monthly intravenous administration of cyclophosphamide. Considering the rapid aggravation of the patient's neurological status, the MRI findings of cortical lesions and the lack of response to any treatment, a biopsy of a frontal lobe lesion was conducted, confirming the presence of confluent, inflammatory-edematous lesions with scattered areas of necrosis and hemorrhage, and ultimately areas of demyelination, thus confirming the diagnosis of AHLE. After more than 5 months of hospitalization the patient was transferred in a primary care facility and remained in a permanent vegetative state until his death, more than 2 years later.

Acute hemorrhagic leukoencephalitis (AHLE), also known as Weston-Hurst syndrome or Hurst disease, is a rare and rapidly progressive form of acute disseminated encephalomyelitis. It is characterized by severe inflammation, hemorrhage, and necrosis within the white matter of the brain. AHLE often follows an upper respiratory infection or other systemic illnesses, suggesting a potential post-infectious autoimmune mechanism. The disease is associated with a high mortality rate and significant disability among survivors. We present the case of a 46-year-old Indian woman with a history of chronic hepatitis B (HBV) who presented with an insidious onset of right-sided limb weakness and bi-frontal headaches. Initial brain MRIs showed features of tumefactive demyelination. Despite aggressive treatment with intravenous (IV) methylprednisolone, IV immunoglobulin, and anti-edema measures, the patient's condition rapidly deteriorated, leading to a diagnosis of AHLE following the emergence of hemorrhagic white matter lesions on repeat MRI. Remarkably, with continued treatment, the patient survived and showed gradual neurological improvement, although she remained significantly debilitated at the time of discharge. AHLE represents one of the most severe forms of demyelinating diseases, often resulting in rapid neurological decline and high mortality. This case highlights the potential link between chronic HBV infection with a high viral load and the onset of AHLE. The patient's recovery underscores the importance of early recognition and aggressive treatment in improving outcomes, even in conditions with traditionally poor prognosis. Clinicians should maintain a high index of suspicion for AHLE in patients with chronic viral infections presenting with neurological symptoms. Prompt and aggressive management can be life-saving, and ongoing research is needed to better understand the pathogenesis and optimal treatment strategies for this rare but devastating condition., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Sivasubramanian et al.)

A 68-year-old Japanese man developed a fever, headache, hiccups, and altered consciousness. Brain magnetic resonance imaging (MRI) revealed a hemorrhagic lesion in the right temporal lobe and multiple high-intensity white matter lesions. A brain biopsy showed pathological findings consistent with acute disseminated encephalomyelitis (ADEM), suggesting a diagnosis of acute hemorrhagic leukoencephalitis (AHLE), an aggressive ADEM variant. The patient also developed myodesopsia and was diagnosed with retinal vasculitis, likely due to a hyperimmune state caused by AHLE. Corticosteroids enabled full recovery. Although AHLE is uncommon in elderly individuals, clinicians should be aware of its occurrence in this patient subgroup and recognize potential retinal manifestations associated with AHLE.