Cranial Neuropathy as Sign of Systemic Vasculitis: Clinical Case
DOI:
https://doi.org/10.60591/crspmi.75Keywords:
Nervous System Diseases, VasculitisAbstract
Vasculitides are a heterogeneous group of diseases, characterized by inflammation and necrosis of the blood vessel walls. Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis is a small vessels vasculitis and include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis.
Vasculitides can be life threatening. Immunosuppression is the cornerstone of therapy.
We present the case of a 55-year-old man who in September of 2020 was evaluated in the emergency department for fever, night sweats, myalgia and asthenia. At the same time he had palpable purpura in both legs and inflammatory, symmetrical and migratory arthralgias. He was admitted to an internal medicine ward and during the hospitalization he developed headache, left peripheral facial paralysis, mild bilateral sensorineural hearing loss and horizontal nystagmus. Blood analysis revealed positive proteinase 3 (PR3) ANCA and raised serum inflammatory markers. The diagnosis of PR3-ANCA positive systemic vasculitis with cranial neuropathies involving the VII and VIII pairs was established and he started rituximab and high dose steroids with complete regression of the systemic symptoms and gradual improvement of the cranial neuropathies.
Although uncommon, cranial neuropathy can be the defining manifestation of ANCA-positive vasculitis, as shown in this clinical case.
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