Department of Neurology, University Hospital 12 de Octubre, Madrid, Spain.
Minerva Med. 2011 Dec;102(6):417-40.
Essential tremor (ET) is one of the most common neurological disorders among adults, and is the most common tremor disorder. ET has classically been viewed as a benign monosymptomatic condition. Yet over the past 10 years, a growing body of evidence indicates that this is a progressive condition that is clinically heterogeneous, and may be associated with a variety of different features such as gait abnormalities, parkinsonism, cognitive impairment, dementia, personality disturbances, depressive symptoms, and sensory abnormalities (e.g., mild olfactory dysfunction and hearing impairment). In addition, postmortem studies are showing a pathologically heterogeneous neurodegenerative disease. The emerging view is that ET might be a family of diseases, unified by the presence of kinetic tremor, but further characterized by etiological, clinical and pathological heterogeneity. The diagnosis of ET is clinical and made by history and physical examination. Effective pharmacological treatments for the disorder currently remain limited. Drugs are generally initiated when the tremor begins to interfere with the patient's ability to perform daily activities or when the tremor becomes embarrassing. For severe, medically refractory ET, thalamic ventralis intermedius nucleus deep brain stimulation may lessen tremor and improve function.
特发性震颤(ET)是成年人中最常见的神经障碍之一,也是最常见的震颤障碍。ET 经典地被视为良性单症状疾病。然而,在过去的 10 年中,越来越多的证据表明,这是一种进行性疾病,临床上具有异质性,可能与各种不同的特征相关,如步态异常、帕金森病、认知障碍、痴呆、人格障碍、抑郁症状和感觉异常(例如,嗅觉轻度障碍和听力障碍)。此外,尸检研究显示出一种病理性异质性神经退行性疾病。新兴观点认为,ET 可能是一组疾病,以运动性震颤为特征,但进一步以病因、临床和病理异质性为特征。ET 的诊断是临床诊断,通过病史和体格检查做出。目前,对该疾病有效的药物治疗仍然有限。当震颤开始干扰患者进行日常活动的能力或当震颤变得尴尬时,通常会开始使用药物。对于严重的、药物难治性 ET,丘脑腹侧中间核深部脑刺激可能会减轻震颤并改善功能。