Defazio G, Conte A, Gigante A F, Ferrazzano G, Pellicciari R, Dagostino S, Fabbrini G, Berardelli A
Department of Basic Science, Neuroscience and Sense Organs, Aldo Moro University of Bari, Italy.
Department of Neurology and Psychiatry, Sapienza University of Rome, Italy; Neuromed Institute IRCCS, Pozzilli, Italy.
Parkinsonism Relat Disord. 2017 Jul;40:64-68. doi: 10.1016/j.parkreldis.2017.04.014. Epub 2017 Apr 25.
Idiopathic blepharospasm is a clinically heterogeneous condition. It is not known whether the various manifestations become manifest sequentially during the course of the disease or aggregate in separate clusters identifying subpopulations of patients.
Eighty-nine patients with idiopathic blepharospasm were assessed using k-means cluster analysis to identify relatively homogeneous groups on the basis of low-intragroup/high-intergroup differences across a set of selected variables.
The results suggest that there may be three groups of patients. Group 1 included patients who had prolonged muscle spasms leading to complete rim closure associated with brief and/or prolonged spasms with incomplete rim closure, the most severe blepharospasm, and a greater tendency to spread to adjacent segments. Group 2 included patients characterized by prolonged spasms with partial rim closure, either alone or associated with brief spasms whereas Group 3 included patients with brief spasms with complete rim closure, the least severe blepharospasm, and the lowest tendency to spread. The severity of Group 2 blepharospasm was between that observed in Group 1 and Group 3, while the tendency to spread was similar to Group 3. The three groups did not differ for disease duration, age of onset, sex and other clinical features. The observation that inhibition of the R2 component of the blink reflex recovery cycle was more abnormal in Groups 1/2 2 than in Group 3 at least in part validates our classification.
The present study suggests that blepharospasm patients may be classified in different subtypes according to the type of spasms, severity of the condition and tendency to spread.
特发性眼睑痉挛是一种临床异质性疾病。尚不清楚各种表现是在疾病过程中依次出现,还是聚集在不同的簇中以识别患者亚群。
对89例特发性眼睑痉挛患者进行k均值聚类分析,以根据一组选定变量的组内差异低/组间差异高来识别相对同质的组。
结果表明可能存在三组患者。第1组包括肌肉痉挛持续时间长导致睑裂完全闭合的患者,伴有睑裂不完全闭合的短暂和/或持续痉挛,最严重的眼睑痉挛,以及扩散到相邻节段的倾向更大。第2组包括以睑裂部分闭合的持续痉挛为特征的患者,单独出现或伴有短暂痉挛,而第3组包括睑裂完全闭合的短暂痉挛患者,眼睑痉挛最不严重,扩散倾向最低。第2组眼睑痉挛的严重程度介于第1组和第3组之间,而扩散倾向与第3组相似。三组在病程、发病年龄、性别和其他临床特征方面无差异。至少部分地,第1/2组眨眼反射恢复周期的R2成分抑制比第3组更异常这一观察结果验证了我们的分类。
本研究表明,眼睑痉挛患者可根据痉挛类型、病情严重程度和扩散倾向分为不同亚型。