Acute hemorrhagic leukoencephalitis: a critical entity for forensic pathologists to recognize

Area of Science:

• Neurology
• Pathology
• Forensic Medicine

Background:

• Acute hemorrhagic leukoencephalopathy (AHLE) is a rare, hyperacute neurological disorder.
• It presents with perivenular demyelination and diffuse hemorrhagic necrosis of the central nervous system.
• AHLE is associated with high morbidity and mortality, often mimicking CNS infections.

Observation:

• A previously healthy 11-year-old boy developed gastroenteritis-like symptoms, progressing to severe headache, dizziness, and focal neurological deficits.
• Head CT revealed thalamic edema; CSF analysis suggested an infectious etiology.
• Despite empiric therapies, the patient's condition rapidly deteriorated, leading to death within 14 days.

Findings:

• Autopsy was requested to determine the proximate cause of death in this case of AHLE.
• AHLE typically presents abruptly after a viral illness or vaccination, with rapid deterioration and poor prognosis.
• The exact cause of AHLE remains unclear, but an autoimmune pathophysiology involving myelin cross-reactivity is suspected.

Implications:

• Forensic pathologists must consider AHLE in differential diagnoses, especially in cases with rapid neurological decline and death.
• Early recognition and potential treatment with immunosuppressants or plasma exchange may offer a therapeutic window, though outcomes remain poor.
• This case underscores the aggressive nature of AHLE and the need for further research into its pathogenesis and treatment.