Late-Onset Narcolepsy with Cataplexy Unresponsive to Intravenous
High-Dose Immunoglobulin Treatment

J Korean Neurol Assoc > Volume 30(1); 2012 > Article

Journal of the Korean Neurological Association 2012;30(1): 57-59.

고농도면역글로불린정맥주사 치료에 무반응을 보인 허탈발작을 동반한 만발성 기면병

조재욱, 정나연 배진원 김대진 이재혁 타카시 칸바야시 a

양산부산대학교병원 신경과, 아키타 의과대학 신경정신과학교실 a

Late-Onset Narcolepsy with Cataplexy Unresponsive to Intravenous High-Dose Immunoglobulin Treatment

Jae-Wook Cho

Department of Neurology, Pusan National University Yangsan Hospital, Medical Research Institute of Pusan National University, Yangsan, Korea Department of Neuropsychiatry a , Akita University School of Medicine, Akita, Japan

Abstract

Narcolepsy with cataplexy (NC) is associated with hypocretin deficiency, and is thought to be an autoimmunity condition. The mean age at onset is estimated to be in the early 20s. Recent papers have addressed the response to immunotherapies in NC, with challenging results. We report a case of late-onset NC in a patient who did not benefit from early intravenous high-dose immunoglobulin (IVIg) therapy. This is the first reported attempt at using IVIg to treat an NC patient in Korea. Key Words: Narcolepsy with cataplexy, Intravenous immunoglobulins