PDF(Pubmed)
Abstract：
Idiopathic cervical dystonia (ICD) is by far the largest subgroup of dystonia. Still, its natural course is largely unknown. We studied the natural course of 100 ICD patients from our botulinum toxin clinics (age at ICD onset 45.8 ± 13.5 years, female/male ratio 2.0) over a period of 17.5 ± 11.5 years with follow-ups during botulinum toxin therapy and with semi-structured interviews. Two courses of ICD could be distinguished by symptom development of more or less than 6 months. ICD-type 2 was less frequent (19% vs 81%, p < 0.001), had a more rapid onset (8.7 ± 8.0 weeks vs 3.8 ± 3.5 years), a higher remission rate (92% vs 5%, p < 0.001) and a higher prevalence of excessive psychological stress preceding ICD (63% vs 1%, p < 0.001). In both ICD-types, the plateau phase was non-progressive. Significant differences in patient age at ICD onset, latency and extent of remission, female/male ratio and prevalence of family history of dystonia could not be detected. ICD is a non-progressive disorder. ICD-type 1 represents the standard course. ICD-type 2 features rapid onset, preceding excessive psychological stress and a high remission rate. These findings will improve prognosis, treatment strategies and understanding of underlying disease mechanisms. They contradict the widespread fear of patients of a constant and continued decline of their condition. Excessive psychological stress may be an epigenetic factor triggering the manifestation of genetically predetermined dystonia.
摘要：
特发性宫颈肌张力障碍（ICD）是迄今为止最大的肌张力障碍亚组。尽管如此，它的自然过程在很大程度上是未知的。我们研究了来自我们的肉毒杆菌毒素诊所的100名ICD患者的自然病程（ICD发病年龄为45.8±13.5岁，女性/男性比率2.0），为期17.5±11.5年，在肉毒杆菌毒素治疗期间进行随访，并进行半结构化访谈。ICD的两个疗程可以通过症状发展多于或少于6个月来区分。ICD-2型频率较低(19%vs81%，p<0.001)，起病更快（8.7±8.0周vs3.8±3.5年），较高的缓解率(92%vs5%，p<0.001)和ICD之前过度心理压力的患病率较高(63%对1%，p<0.001)。在两种ICD类型中，平台期是非进行性的。ICD发病时患者年龄存在显著差异，潜伏期和缓解程度，无法检测到肌张力障碍的女性/男性比例和家族史。ICD是非进行性障碍。ICD-type1代表标准课程。ICD-2型起病迅速,之前过度的心理压力和高缓解率。这些发现将改善预后，治疗策略和对潜在疾病机制的理解。它们与患者对病情持续和持续下降的普遍恐惧相矛盾。过度的心理压力可能是引发遗传预先确定的肌张力障碍表现的表观遗传因素。
