Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", Neuroscience Section, University of Salerno, Via Allende 43, 84081, Baronissi, SA, Italy.
Neurology Unit, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
Neurol Sci. 2022 Sep;43(9):5369-5376. doi: 10.1007/s10072-022-06104-w. Epub 2022 May 24.
The recently released classification has revised the nosology of tremor, defining essential tremor (ET) as a syndrome and fueling an enlightened debate about some newly conceptualized entities such as ET-plus. As a result, precise information of demographics, clinical features, and about the natural history of these conditions are lacking.
The ITAlian tremor Network (TITAN) is a multicenter data collection platform, the aim of which is to prospectively assess, according to a standardized protocol, the phenomenology and natural history of tremor syndromes.
In the first year of activity, 679 patients have been recruited. The frequency of tremor syndromes varied from 32% of ET and 41% of ET-plus to less than 3% of rare forms, including focal tremors (2.30%), task-specific tremors (1.38%), isolated rest tremor (0.61%), and orthostatic tremor (0.61%). Patients with ET-plus were older and had a higher age at onset than ET, but a shorter disease duration, which might suggest that ET-plus is not a disease stage of ET. Familial aggregation of tremor and movement disorders was present in up to 60% of ET cases and in about 40% of patients with tremor combined with dystonia. The body site of tremor onset was different between tremor syndromes, with head tremor being most commonly, but not uniquely, associated with dystonia.
The TITAN study is anticipated to provide clinically relevant prospective information about the clinical correlates of different tremor syndromes and their specific outcomes and might serve as a basis for future etiological, pathophysiological, and therapeutic research.
最近发布的分类修订了震颤的分类学,将特发性震颤(ET)定义为一种综合征,并引发了关于一些新构想实体(如 ET-plus)的启发性辩论。因此,这些疾病的人口统计学、临床特征和自然史的准确信息仍然缺乏。
意大利震颤网络(TITAN)是一个多中心数据收集平台,其目的是根据标准化方案前瞻性评估震颤综合征的表现和自然史。
在活动的第一年,已经招募了 679 名患者。震颤综合征的频率从 ET 的 32%和 ET-plus 的 41%到包括局灶性震颤(2.30%)、任务特异性震颤(1.38%)、孤立性静止性震颤(0.61%)和体位性震颤(0.61%)在内的罕见形式不到 3%。ET-plus 患者年龄较大,发病年龄高于 ET,但病程较短,这可能表明 ET-plus 不是 ET 的疾病阶段。震颤和运动障碍的家族聚集在高达 60%的 ET 病例和约 40%的震颤合并肌张力障碍的患者中存在。震颤起始的身体部位在不同的震颤综合征之间有所不同,头部震颤虽然最常见,但并非唯一与肌张力障碍相关。
TITAN 研究预计将提供有关不同震颤综合征的临床相关性及其特定结局的临床相关前瞻性信息,并可能成为未来病因、病理生理学和治疗研究的基础。