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Clinical characterization and successful treatment of 6 patients with Churg-Strauss syndrome-associated neuropathy

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Title: Clinical characterization and successful treatment of 6 patients with Churg-Strauss syndrome-associated neuropathy
Authors: Nakamura, Masakazu Browse this author
Yabe, Ichiro Browse this author →KAKEN DB
Yaguchi, Hiroaki Browse this author
Kishimoto, Riichiro Browse this author
Mito, Yasunori Browse this author
Fujiki, Naoto Browse this author
Houzen, Hideki Browse this author
Tsuji-Akimoto, Sachiko Browse this author
Niino, Masaaki Browse this author
Sasaki, Hidenao Browse this author
Keywords: Churg-Strauss syndrome
conduction block
intravenous immunoglobulin
Issue Date: Oct-2009
Publisher: Elsevier
Journal Title: Clinical Neurology and Neurosurgery
Volume: 111
Issue: 8
Start Page: 683
End Page: 687
Publisher DOI: 10.1016/j.clineuro.2009.07.004
PMID: 19647930
Abstract: Objective: To confirm the reported findings and clarify unknown clinical features of Churg-Strauss syndrome (CSS) -associated neuropathy and design appropriate treatment. Patients and Methods: We assessed the clinical features of 6 patients with CSS-associated neuropathy. Results: Mononeuritis multiplex was present in 4 cases and polyneuropathy in the remaining cases. Both groups progressed to sensori-motor polyneuropathy in an acute or subacute course. All cases showed bronchial asthma and eosinophilia. Two cases with serum antineutrophil cytoplasmic antibodies to myeloperoxidase (MPO-ANCA) had an acute clinical course and severe symptoms. Nerve conduction studies (NCS) of these 2 cases revealed conduction blocks at the initial stage, although NCS finally indicated sensori-motor axonopathy at the involved extremities. For treatment, high-dose corticosteroid therapy for 4 cases, and cyclophosphamide combined with corticosteroids for one case, were effective. For the remaining case, intravenous immunoglobulin (IVIg) at the chronic phase resulted in a slow improvement of neuropathy in the symptomatic aspect. There was no relapse of neuropathy with low dose corticosteroid treatment for 14-24 months after the initial treatment, except one case. There was also no relapse in the other case that was treated with moderate-dose steroids. Conclusion: Our study showed that CSS-associated neuropathy is a treatable disorder and that the first choice therapy is high-dose corticosteroid. In cases where corticosteroids are ineffective or for severe cases, immunosuppressive therapy (cyclophosphamide) with steroids should be considered, and IVIg might be a treatment option.
Type: article (author version)
Appears in Collections:医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 矢部 一郎

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