Severe Axonal Polyneuropathy Revealing Eosinophilic Granulomatosis With Polyangiitis: A Case Report and Review of the Literature

重度轴索性多发性神经病伴嗜酸性肉芽肿性血管炎:病例报告及文献复习

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Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic small- and medium-vessel vasculitis characterized by asthma, hypereosinophilia, and multisystem involvement. Peripheral neuropathy is a recognized neurological manifestation of EGPA and may occasionally present as severe axonal polyneuropathy. We report the case of a 64-year-old male with a 10-year history of asthma who presented with progressive lower limb weakness and paresthesia over three months, which led to wheelchair dependence. Electroneuromyography (ENMG) confirmed a severe axonal sensorimotor polyneuropathy. Laboratory findings showed marked hypereosinophilia (9,030/mm³), strongly positive antineutrophil cytoplasmic antibodies (ANCA), and elevated inflammatory markers. Thoraco-abdominopelvic CT revealed bilateral interstitial and alveolar infiltrates. The diagnosis of EGPA was established according to the American College of Rheumatology (ACR) criteria. Treatment with corticosteroids and cyclophosphamide resulted in significant neurological improvement. This report emphasizes the importance of considering EGPA in asthmatic patients presenting with severe axonal polyneuropathy. Early diagnosis and immunosuppressive therapy are essential to improve outcomes.

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