摘要
目的研究多巴反应性肌张力障碍患者的临床特点。方法对16例家族性患者和10例散发性多巴反应性肌张力障碍患者进行病史采集、神经系统体格检查和CT或MRI检查,所得数据应用SPSS13.0软件进行统计分析。结果26例患者,男10例,女16例;发病年龄为4～48岁,平均(17.46±12.95)岁。(1)首发症状:儿童期(12例)和青春期(6例)发病患者多以肌张力障碍为首发症状,成年期(8例)发病者则以帕金森综合征或肌张力障碍发病。(2)症状波动性:儿童期组和青春期组患者症状均呈现日间波动性,成年期组仅4例症状具有日间波动性,3组相比差异具有显著性意义(χ2=10.227,P=0.006);而且症状波动性与年龄呈负相关(r=#0.715,P<0.01)。(3)姿势性震颤:3组患者姿势性震颤的发生率差异具有显著性意义(χ2=8.073,P=0.018),其中以儿童期发病者发生率最低(5/12),成年期发生率最高(8/8);姿势性震颤的发生与患者年龄呈正相关(r=0.483,P=0.012)。(4)腱反射:3组间腱反射亢进发生率差异无显著性意义(χ2=5.303,P=0.071),腱反射亢进发生率与年龄呈负相关(r=#0.356,P=0.044)。(5)药物反应:26例患者均对左旋多巴治疗有明显而持续的反应。结论多巴反应性肌张力障碍临床表现呈多样化,临床表现与年龄密切相关。左旋多巴对多巴反应性肌张力障碍患者可产生明显而持续的疗效。
Objective To study the clinical characteristics of patients with dopa-responsive dystonia （DRD）. Methods Sixteen familiar DRD patients and 10 patients with sporadic DRD were studied by history taking, physical examination of nervous system and CT/MR scaning. The obtained data were statistical analyzed with SPSS 13.0 software in computer. Results The mean onset age of the patients （10 males and 16 females） was （17.46±12.95） years. Most of patients with onset in childhood （n = 12） and adolescence （n = 6） started with dystonia. Those with onset in adulthood （n = 8） started with Parkinsonism or dystonia. The symptoms and signs presented with marked diurnal fluctuation in childhood and adolescence （100%）, but only 4 adult patients （50%） had diurnal variation. There were significant differences among the three groups （X^2= 10.227, P=0.006）. The diurnal fluctuation was negatively correlated with age （r= -0.715, P〈0.01）. The differences in incidence rate of postural tremor were also found among the 3 groups （X^2= 8.073, P= 0.018）. The incidence was highest in adult group （8/8） and lowest in child group （5/12）. The pastural tremor was positively correlated with age （r= 0.483, P= 0.012）. There were no differences in incidence of tendon hyperreflexia among the three groups （X2=5.303, P=0.071）, and the incidence of tendon hyperreflexia was negatively correlated with age （r = - 0.356, P= 0.044）. All of the 26 cases showed marked and sustained therapeutic effect on L-Dopa. Conclusion The clinical manifestations of DRD are various and they are closely correlated with age. It demonstrates that all patients markedly and sustainly respond to L-Dopa.
出处
《中国现代神经疾病杂志》
CAS
2006年第2期114-118,共5页
Chinese Journal of Contemporary Neurology and Neurosurgery
