Zilber N, Inzelberg R, Kahana E, Korczyn A D
Centre de Recherche Français de Jérusalem, UMR CNRS-DGRCST, Israel.
Neuroepidemiology. 1994;13(5):195-201. doi: 10.1159/000110379.
The natural history of 51 idiopathic torsion dystonia cases identified in a nationwide survey in Israel was studied. In order to take advantage of all data regarding the evolution of the disease, independently of the length of follow-up, we have used a life table method of analysis. An estimate of the mean degree of generalization of the disease at different times from onset was calculated and a curve of the estimated temporal evolution was drawn. This analysis confirmed the heterogeneity of the disease course. The most rapid deterioration occurred in patients with juvenile onset in the lower limbs, particularly in the first 2 years following the onset. The rate of evolution was not influenced by gender or familiarity. The course of the disease seemed more deleterious in non-Ashkenazic Jews. The present statistical method of estimating the evolution in different patient groups should be useful especially for the evaluation of therapeutic interventions.
对以色列全国性调查中确诊的51例特发性扭转性肌张力障碍病例的自然病史进行了研究。为了利用所有与疾病进展相关的数据,而不考虑随访时间的长短,我们采用了寿命表分析方法。计算了自发病起不同时间疾病平均泛化程度的估计值,并绘制了估计的时间演变曲线。该分析证实了疾病病程的异质性。最快速的恶化发生在青少年起病且累及下肢的患者中,尤其是在发病后的头两年。疾病进展速度不受性别或家族史影响。在非阿什肯纳兹犹太人中,疾病病程似乎更具危害性。目前用于估计不同患者群体病情演变的统计方法,尤其对治疗干预措施的评估应是有用的。