Neurological disorders in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

• Published in: Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova Vol. 116; no. 10; p. 93
• Main Authors: Gilyarov, M Yu, Belikova, L P, Shchukin, I A, Fidler, M S, Avakyan, G G, Shustrova, G E, Martynov, M Yu, Svet, A V
• Format: Journal Article
• Language: Russian
• Published: Russia (Federation) 2016
• Subjects: eosinophilic granulomatosis with polyangiitis; Churg—Strauss syndrome; ANCA; vasculitis; venous sinus thrombosis; polyneuropathy; eosinophilia; neuropathy; stroke
• ISSN: 1997-7298
• DOI: 10.17116/jnevro201611610193-102

Eosinophilic granulomatosis with polyangiitis - EGPA (Churg-Strauss syndrome) is a rare autoimmune disorder. The pathogenesis of the disease includes production of anti-neutrophil cytoplasmic antibodies directed against myeloperoxidase with the development of small-vessel necrotizing vasculitis and eosinophilic infiltration of organs. The involvement of peripheral and central nervous system is observed in more than 3/4 of cases. The authors describe three patients with EGPA. In a 53-year-old male patient, EGPA manifested with multiple neuropathies, which regressed after treatment with corticosteroids and cytostatics. In a 34-year-old woman, cerebral sinus thrombosis and cerebral infarction developed in the non-active period of long-term EGPA. The patient was treated with anticoagulants. A 77-year-old woman with a newly diagnosed EGPA, confirmed by bone marrow examination for eosinophilia, developed ischemic stroke and polyneuropathy. The causes and mechanisms of development as well as dynamics and outcomes of neurological disorders, differential diagnosis, treatment and prognosis of eosinophilic granulomatosis with polyangiitis are discussed.