Neurologic Manifestations of Churg-Strauss Syndrome

J Korean Neurol Assoc > Volume 17(6); 1999 > Article

Journal of the Korean Neurological Association 1999;17(6): 836-840.

척-스트라우스 증후군의 신경학적 발현

강동화, 정인경*·김호진 ·이광우

서울대학교 의과대학 신경과학교실,내과학교실*

Neurologic Manifestations of Churg-Strauss Syndrome

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

Departments of Neurology and Internal Medicine*, Seoul National University, College of Medicine

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. J Korean Neurol Assoc 17(6):836~840, 1999 Key Words : Churg-Strauss syndrome, Neurologic manifestation, Peripheral neuropathy, Cerebral infarction