Neurologic Manifestations of Churg-Strauss Syndrome

Kang, Dong Wha; Jeong, In Kyong; Kim, Ho Jin; Lee, Kwang Woo

Abstract

BACKGROUND: Churg-Strauss syndrome (CSS) is characterized by disseminated vasculitis with multi-organ involve-ment. The purpose of this study was to determine the frequency and the types of neurologic involvement in a series of patients with CSS.
METHODS
We reviewed the medical records of 16 patients (seven men and nine women, age = 41.9 (18.6) with CSS who were examined at Seoul National University Hospital. The diagnosis of CSS was based on the presence of asthma, peripheral eosinophilia (more than 10% eosinophiles), and histopathological or clinical findings of vasculitis.
RESULTS
Of the 16 patients, 12 (75%) had neurologic involvement. Nine (56%) had neurologic symptoms as initial presentations of CSS. Peripheral neuropathy was detected in nine patients (56%) ; six had multiple mononeu-ropathy, and three had distal symmetric polyneuropathy. Three patients (19%) had cerebral infarctions; in two of them, a delayed diagnosis of CSS caused the recurrence of ischemic stroke. Corticosteroid therapy combined with immuno-suppressive agents usually yielded improvement or stabilization of symptoms.
CONCLUSIONS
Neurologic involvement is common in CSS, usually manifesting as peripheral neuropathy. Neurologic symptoms are important initial manifesta-tions at the time of diagnosis of CSS. Furthermore, cerebral involvement is not uncommon; thus any neurologic symp-toms in patients with asthma or eosinophilia prompts an aggressive diagnostic approach to CSS.