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The natural course of idiopathic cervical dystonia
Journal of Neural Transmission ( IF 3.3 ) Pub Date : 2024-01-20 , DOI: 10.1007/s00702-023-02736-0
Dirk Dressler , Bruno Kopp , Lizhen Pan , Fereshte Adib Saberi

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.



中文翻译:

特发性颈肌张力障碍的自然病程

特发性颈肌张力障碍(ICD)是迄今为止最大的肌张力障碍亚组。尽管如此,其自然进程在很大程度上还是未知的。我们研究了来自我们肉毒毒素诊所的 100 名 ICD 患者(ICD 发病年龄 45.8 ± 13.5 岁,女性/男性比例 2.0)的自然病程,历时 17.5 ± 11.5 年,并在肉毒毒素治疗期间和半治疗期间进行随访。结构化面试。两个 ICD 疗程可通过症状发展时间长于或短于 6 个月来区分。ICD 2 型发生频率较低(19% vs 81%,p  < 0.001),起效更快(8.7 ± 8.0 周 vs 3.8 ± 3.5 年),缓解率较高(92% vs 5%,p  < 0.001) )以及 ICD 之前过度心理压力的发生率较高(63% vs 1%,p  < 0.001)。在两种 ICD 类型中,平台期都是非进行性的。无法检测到 ICD 发病时患者年龄、潜伏期和缓解程度、女性/男性比例以及肌张力障碍家族史患病率的显着差异。ICD 是一种非进行性疾病。ICD 类型 1 代表标准课程。ICD-2型具有起病快、心理压力过大、缓解率高的特点。这些发现将改善预后、治疗策略和对潜在疾病机制的理解。它们与患者普遍担心病情持续恶化的恐惧相矛盾。过度的心理压力可能是引发遗传预定肌张力障碍表现的表观遗传因素。

更新日期:2024-01-21
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