NEUROLOGIC MANIFESTATIONS OF CHURG-STRAUSS-SYNDROME

Objective: To determine the frequency and the types of neurologic invo lvement in a series of patients with Churg-Strauss syndrome (CSS). Des ign: We reviewed the medical records of 47 consecutive patients with C SS who were examined at the Mayo Clinic between January 1974 and June 1992. Material and Methods: The study patients were classified into tw o groups: (1) those with a histopathologically confirmed diagnosis of CSS who had evidence of either vasculitis of Churg-Strauss granuloma, the presence of asthma, and peripheral eosinophilia (more than 10% eos inophils) on at least one differential leukocyte count (N=33) and (2) those with a clinical diagnosis of CSS who had evidence of vasculitis based on either multiple mononeuropathy or necrotizing cutaneous lesio ns, the presence of asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N=14). Res ults: Of the 47 patients, 29 (62%) had neurologic involvement. Periphe ral neuropathy was detected in 25 patients: 17 had multiple mononeurop athy, 7 had distal symmetric polyneuropathy. Three patients had cerebr al infarctions. Less commonly identified problems included radiculopat hies, ischemic optic neuropathy, and bilateral trigeminal neuropathy. Asthma preceded the onset of neurologic involvement in all cases (mean duration, 6.7 years). Follow-up data, when available, showed that cor ticosteroid therapy usually yielded improvement or stabilization. Conc lusion: Neurologic involvement is common in CSS, usually manifesting a s peripheral neuropathy. In this series of patients, asthma preceded t he neurologic manifestations.