大剂量静脉内注射免疫球蛋白治愈硬化性黏液水肿伴发的痴呆
Dementia associated with scleromyxoedema reversed by high- dose intravenous immunoglobulin作者机构：Department of Dermatology Royal Free Hospital London NW3 2QG United Kingdom
出 版 物：《世界核心医学期刊文摘（皮肤病学分册）》 (Digest of the World Core Medical JOurnals:Dermatology)
年 卷 期：2005年第1卷第12期
页 面：53-53页
学科分类：1002[医学-临床医学] 100206[医学-皮肤病与性病学] 10[医学]
主 题：硬化性黏液水肿 静脉内注射 免疫球蛋白 智能状态 酸性黏多糖 脑病 认知缺陷 风湿症 病主
摘 要：Scleromyxoedema is a rare skin disease, characterized by deposition of acid mucopolysaccharides in the dermis. Although the disease primarily affects the skin, cardiovascular, renal and rheumatological manifestations have been described. In addition to these noncutaneous manifestations, about 15% of patients have central neurological symptoms such as psychosis, convulsions and encephalopathy. Successful therapy is difficult but high- dose intravenous immunoglobulin (IVIg) has been reported to be a successful treatment. We describe a patient with scleromyxoedema who presented with novel central nervous system manifestations of chronic cognitive impairment and dementia (Folstein Mini Mental State test score 8/30), which improved within a week after treatment with high- dose IVIg, with full restoration (Folstein Mini Mental State test score 27/30) at 2 months.
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