Department of Neurology, Emory University, Atlanta, GA, USA.
J Neurol Sci. 2013 Aug 15;331(1-2):98-101. doi: 10.1016/j.jns.2013.05.022. Epub 2013 Jun 6.
Blepharospasm is a form of focal dystonia that manifests as repetitive involuntary closure of the eyes. The pathogenesis of blepharospasm and the neuroanatomic substrates involved are not fully understood. Dysfunction of the basal ganglia traditionally is presumed to be the main cause of most forms of dystonia, but a growing body of evidence suggests that a network of additional cortical and subcortical structures may be involved.
The medical records of 1114 patients with blepharospasm seen over past 10 years at Emory University were reviewed to identify potentially contributing brain lesions. A systematic review of the published literature was also conducted to identify potentially contributing brain lesions.
Among patients with blepharospasm at Emory University, 18 had focal lesions on imaging studies available for review. The literature review revealed 25 articles describing 30 additional cases of blepharospasm associated with focal lesions. Among all 48 cases, lesions were found in multiple regions including the thalamus (n=12), lower brainstem (n=11), basal ganglia (n=9), cerebellum (n=9), midbrain (n=7), and cortex (n=1).
These data in combination with functional imaging studies of primary blepharospasm support a model in which a network of different regions plays a role in the pathogenesis of blepharospasm.
眼睑痉挛是一种局灶性肌张力障碍，表现为眼睛反复无意识地闭合。眼睑痉挛的发病机制和涉及的神经解剖学基础尚未完全清楚。基底节功能障碍传统上被认为是大多数形式的肌张力障碍的主要原因，但越来越多的证据表明，一个额外的皮质和皮质下结构网络可能也与之相关。
回顾了过去 10 年在埃默里大学就诊的 1114 例眼睑痉挛患者的病历，以确定可能导致病变的脑部病变。还对已发表的文献进行了系统综述，以确定可能导致病变的脑部病变。
在埃默里大学的眼睑痉挛患者中，有 18 例在可用于复查的影像学研究中有局灶性病变。文献综述发现了 25 篇描述 30 例与局灶性病变相关的额外眼睑痉挛病例的文章。在所有 48 例病例中，病变位于多个区域，包括丘脑（n=12）、下脑桥（n=11）、基底节（n=9）、小脑（n=9）、中脑（n=7）和皮质（n=1）。
这些数据结合原发性眼睑痉挛的功能影像学研究，支持这样一种模型，即不同区域的网络在眼睑痉挛的发病机制中起作用。
