Clinical and Electrophysiologic Features of Peripheral Neuropathy in Churg-Strauss Syndrome

ABSTRACT

BACKGROUND: Churg-Strauss syndrome (CSS) is characterized by disseminated vasculitis and eosinophilia in patients with asthma. This study was performed to delineate the characteristics of peripheral neuropathy and to identify the factors associated with the severity of neurologic disability. METHODS: We reviewed the medical records of 12 consecutive patients with Churg-Strauss syndrome who had been diagnosed at Samsung Medical Center. The diagnosis of CSS was based on the American college of Rheumatology 1990 criteria. RESULTS: Of the 12 patients, 9 had neurologic involvement. Six had neurologic symptoms as presenting manifestations. All neurologic manifestations were peripheral neuropathy; 5 had multiple mononeuropathy, 2 had distal symmetric polyneuropathy, and 2 had mononeuropathy. Neurologic disability score depended on the rapidity of steroid treatment. CONCLUSIONS: Neurologic involvement is common and develops early in patients with CSS, usually manifesting as peripheral neuropathy. Neurologic disability score showed that early steroid therapy was related with less neurologic disability.