舞蹈病-棘红细胞增多症合并慢性活动性肝炎的临床特点(附1例报告)  

Clinical features of chorea-acanthocytosis complicated with chronic active hepatitis( report of 1 case)

作　　者：栾海辉[1] 刘艺鸣[1] 张建园[1] 陈良[1] 厉郡华 张孝国[3] 

机构地区：[1]山东大学齐鲁医院神经内科,济南250012 [2]山东省日照市东港区医院神经内科 [3]山东省济南市传染病医院肝病一科

出　　处：《临床神经病学杂志》2013年第5期372-374,共3页Journal of Clinical Neurology

摘　　要：目的探讨舞蹈病-棘红细胞增多症合并慢性活动性肝炎的临床特点。方法回顾性分析1例舞蹈病-棘红细胞增多症合并慢性活动性肝炎患者的临床资料。结果本例患者临床表现包括抑郁,典型的舌及下颌肌张力障碍,进食诱发的吐舌样症状;检查发现乙肝病毒表面抗原(+),血肌酸激酶、乙肝病毒DNA定量及血氨升高;颅脑MRI示尾状核头萎缩,外周血涂片示棘红细胞增多。曾误诊为肝性脑病。经保肝治疗,舞蹈病症状未见明显改善,但肝功能、血氨等均恢复正常。其后随访6个月,复查外周血涂片棘红细胞仍增多。临床确诊为舞蹈病-棘红细胞增多症,乙型肝炎。结论舞蹈病-棘红细胞增多症合并慢性活动性肝炎的临床表现复杂,需与肝性脑病鉴别。Objective To investigate the clinical features of chorea-acanthocytosis complicated with chronic active hepatitis. Methods The clinical date of a chorea-acanthocytosis patient complicated with chronic active hepatitis was analyzed retrospectively. Results The clinical characteristics of this case were mental depression, typical tongue and jaw oromandibular dystonia, eating induced tongue protrusion symptoms. Examinations showed that the hepatitis B virus surface antigen was （ ＋ ）, and the serum creatine kinase, hepatitis B virus DNA copies and blood ammonia were increased. Brain MRI showed atrophy in the head of caudate nucleus. The peripheral blood smear showed that acanthocyte increased. Formerly it was misdiagnosed as hepatic encephalopathy. After protecting the liver, the chorea symptoms were not improved, but the liver function and blood ammonia were recovered. After 6 months follow up, the peripheral blood smear still showed increased acanthocyte. Then it was clinically diagnosed as chorea-acanthocytosis, hepatitis B. Conclusions The clinical characteristics of chorea-acanthocytosis complicated with chronic active hepatitis are intricate. It needs to be distinguished with the hepatic encephalopathy.

关 键 词：舞蹈病-棘红细胞增多症 慢性活动性肝炎 临床特点 

分 类 号：R742.2[医药卫生—神经病学与精神病学]