Atypical Neurological Presentation of Eosinophilic Granulomatosis with Polyangiitis: Concurrent Quadriparesis and Refractory Seizures as Initial Manifestations

Abstract

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a small to medium-vessel vasculitis characterized by asthma, hyper eosinophilia, and multisystem involvement. Neurological disease most frequently manifests as mononeuritis multiplex, whereas symmetric polyradiculopathy and central nervous system (CNS) vasculitis are distinctly uncommon. Case Presentation: We report a 40-year-old woman with long-standing asthma who presented with a rapidly progressive ascending quadriparesis, glove-and-stocking sensory loss and vasculitic rash, she subsequently experienced focal seizures that evolved into refractory status epilepticus. Investigations revealed marked eosinophilia, axonal sensorimotor polyradiculopathy on nerve conduction studies, and a skin biopsy demonstrating eosinophilic vasculitis. Cytogenetic analyses excluded clonal hyper eosinophilic syndromes. MRI of the brain revealed multifocal acute infarcts consistent with CNS vasculitis. ANCA testing was negative. The constellation of asthma, hyper eosinophilia, neuropathy, extravascular eosinophilic inflammation, multisystem involvement and ANCA being negative, fulfilled ACR/EULAR classification criteria for EGPA after excluding clonal HES.  Despite initiation of high-dose corticosteroids and cyclophosphamide, she developed aspiration pneumonia during prolonged seizures and mechanical ventilation and ultimately died from intractable status epilepticus. Conclusion: This case exemplifies an under-recognized neurological spectrum of EGPA, where simultaneous peripheral and central nervous system involvement results in rapid clinical deterioration and fatal outcome. Early recognition of this rare and aggressive variant is crucial to improving therapeutic opportunities and preventing catastrophic progression.