Human Motor Control Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, United States.
Int Rev Neurobiol. 2022;163:103-128. doi: 10.1016/bs.irn.2022.02.004. Epub 2022 Mar 21.
Essential tremor (ET) is one of the most common movement disorders, yet we do not have a complete understanding of its pathophysiology. From a phenomenology standpoint, ET is an isolated tremor syndrome of bilateral upper limb action tremor with or without tremor in other body locations. ET is a pathological tremor that arises from excessive oscillation in the central motor network. The tremor network comprises of multiple brain regions including the inferior olive, cerebellum, thalamus, and motor cortex, and there is evidence that a dynamic oscillatory disturbance within this network leads to tremor. ET is a chronic disorder, and the natural history shows a slow progression of tremor intensity with age. There are reported data suggesting that ET follows the disease model of a neurodegenerative disorder, however whether ET is a degenerative or electrical disorder has been a subject of debate. In this chapter, we will review cumulative evidence that ET as a syndrome is a fundamentally electric disorder. The etiology is likely heterogenous and may not be primarily neurodegenerative.
特发性震颤（ET）是最常见的运动障碍之一，但我们对其病理生理学仍缺乏全面的了解。从现象学的角度来看，ET 是一种孤立的震颤综合征，表现为双侧上肢动作性震颤，或伴有其他身体部位的震颤。ET 是一种病理性震颤，源于中枢运动网络的过度振荡。震颤网络包括多个脑区，包括橄榄核、小脑、丘脑和运动皮层，有证据表明该网络内的动态振荡干扰会导致震颤。ET 是一种慢性疾病，其自然病史显示随着年龄的增长，震颤强度逐渐加重。有报道称，ET 符合神经退行性疾病的疾病模型，但 ET 是退行性疾病还是电紊乱性疾病一直存在争议。在本章中，我们将回顾累积的证据，表明 ET 作为一种综合征，从根本上说是一种电紊乱性疾病。其病因可能是异质的，可能不是主要的神经退行性疾病。
