Valls-Sole Josep, Defazio Giovanni
EMG and Motor Control Section, Neurology Department, Hospital Clinic, University of Barcelona , Barcelona , Spain.
Department of Basic Medical Sciences, Neurosciences and Sensory Organs, "Aldo Moro" University of Bari , Bari , Italy.
Front Neurol. 2016 Mar 31;7:45. doi: 10.3389/fneur.2016.00045. eCollection 2016.
Blepharospasm (BSP) is a rather distressing form of focal dystonia. Although many aspects of its pathophysiological mechanisms are already known, we lack fundamental evidence on etiology, prevention, and treatment. To advance in our knowledge, we need to review what is already known in various aspects of the disorder and use these bases to find future lines of interest. Some of the signs observed in BSP are cause, while others are consequence of the disorder. Non-motor symptoms and signs may be a cue for understanding better the disease. Various cerebral sites have been shown to be functionally abnormal in BSP, including the basal ganglia, the cortex, and the cerebellum. However, we still do not know if the dysfunction or structural change affecting these brain regions is cause or consequence of BSP. Further advances in neurophysiology and neuroimaging may eventually clarify the pathophysiological mechanisms implicated. In this manuscript, we aim to update what is known regarding epidemiology, clinical aspects, and pathophysiology of the disorder and speculate on the directions of research worth pursuing in the near future.
眼睑痉挛(BSP)是一种相当令人苦恼的局灶性肌张力障碍形式。尽管其病理生理机制的许多方面已经为人所知,但我们在病因、预防和治疗方面缺乏基本证据。为了增进我们的认识,我们需要回顾该疾病各个方面的已知情况,并以此为基础找到未来的研究方向。在BSP中观察到的一些体征是病因,而其他一些则是该疾病的后果。非运动症状和体征可能是更好地理解该疾病的线索。已证明在BSP中多个脑区功能异常,包括基底神经节、皮层和小脑。然而,我们仍然不知道影响这些脑区的功能障碍或结构变化是BSP的病因还是后果。神经生理学和神经影像学的进一步进展最终可能会阐明其中涉及的病理生理机制。在本手稿中,我们旨在更新关于该疾病的流行病学、临床方面和病理生理学的已知情况,并推测在不久的将来值得追求的研究方向。