Rapidly progressing idiopathic neutrophilic vasculitis with fibrinoid necrosis of the central nervous system

Rapid neurologic and clinical decline can have a vast differential diagnosis. In this unique case, a man in his mid-70s was found unresponsive at home and progressed to brain death within 36 hours from the initial presentation. Preliminary imaging revealed a dorsal pontine haemorrhage, and later, studies showed additional areas with haemorrhage including parenchyma, midbrain and pons. Other than respiratory failure, he did not demonstrate any other end organ dysfunction before losing remaining brain stem reflexes and expiring despite aggressive efforts. After full-body autopsy, microscopic examination of the brain revealed severe neutrophilic vasculitis with fibrinoid necrosis, most severely affecting the brainstem and cerebellum. This phenomenon was not observed anywhere else in the body. Post-mortem analysis for various infectious causes did not reveal a definitive aetiology. Despite extensive workup, this case does not fit criteria for any established disease entity, and we believe it likely represents an idiopathic, unique disease.