Multiple cranial neuropathies in a patient with eosinophilic granulomatosis with polyangiitis

Abstract

Summary Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis characterised by asthma, eosinophilia and sinonasal disease. Although peripheral neuropathy is common, central nervous system involvement occurs in only 3%–5% of patients and may cause significant morbidity. We present an older adult male with antineutrophil cytoplasmic antibody-negative EGPA, that relapsed after discontinuing mepolizumab and developed sequential cranial neuropathies involving cranial nerves V, VII, II and X, causing trigeminal neuropathy, bilateral facial palsy, optic neuropathy and vocal cord dysfunction. MRI demonstrated hypertrophic pachymeningitis (HP) with multifocal pachymeningeal enhancement. The patient received pulse intravenous methylprednisolone followed by cyclophosphamide infusion, resulting in headache resolution, stabilisation of visual symptoms, improvement in hoarseness and decreased pachymeningeal enhancement on follow-up MRI. He was transitioned to methotrexate and monthly mepolizumab maintenance therapy. This case highlights a rare presentation of EGPA with sequential multiple cranial neuropathies secondary to HP, emphasising the importance of early recognition and aggressive immunosuppressive therapy.