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肌萎缩侧索硬化症中的原始反射:患病率及其相关因素

Primitive reflexes in amyotrophic lateral sclerosis: prevalence and correlates.

作者信息

Tremolizzo Lucio, Susani Emanuela, Lunetta Christian, Corbo Massimo, Ferrarese Carlo, Appollonio Ildebrando

机构信息

Neurology Unit, ALS center and Memory Clinic, San Gerardo Hospital, via Pergolesi 33, Monza, 20900, Italy,

出版信息

J Neurol. 2014 Jun;261(6):1196-202. doi: 10.1007/s00415-014-7342-7. Epub 2014 Apr 13.

Abstract

Identifying frontal impairment in ALS is an important goal albeit disease-dedicated tools are still scarce. For this reason, we decided to consider primitive reflexes (PRs), variably regarded as correlates of frontal release and/or of upper motor neuron (UMN) impairment, often in the setting of dementias. Specifically, the aims of this work consisted in assessing the exact prevalence of the combination of seven PRs in ALS, trying to clarify their role as putative proxies of cognitive impairment or of UMN dysfunction. In this cross-sectional study, 50 consecutive ALS outpatients were evaluated for the presence of: palmomental (PM), corneomandibular (CM), glabella tap (MY), rooting, sucking, snout, and grasping reflexes. Cognitive screening was performed by the Frontal Assessment Battery (FAB) and the Weigl's Sorting test (WST); UMN dysfunction was concomitantly evaluated. PM, CM and MY were more frequently detected (62, 52, and 44 % of the ALS sample, respectively), while the other reflexes were under-represented. Patients displaying three or more PRs had significantly lower FAB and WST scores. On the other hand, UMN dysfunction was only moderately associated to PRs. In conclusion, PRs' assessment is a promising complementary tool for screening cognitive impairment in ALS; however, further work will be necessary to establish its added value with respect to already existing ALS-dedicated screening tools for cognition.

摘要

尽管针对肌萎缩侧索硬化症(ALS)的专用检测工具仍然匮乏,但识别ALS患者的额叶功能障碍仍是一个重要目标。因此,我们决定考虑原始反射(PRs),在痴呆症背景下,PRs常被视作为额叶释放和/或上运动神经元(UMN)功能障碍的相关指标。具体而言,本研究旨在评估ALS患者中七种PRs组合的确切患病率,试图阐明它们作为认知障碍或UMN功能障碍假定替代指标的作用。在这项横断面研究中,对50例连续的ALS门诊患者进行了以下检查:掌颏反射(PM)、角膜下颌反射(CM)、眉间轻叩反射(MY)、觅食反射、吸吮反射、噘嘴反射和抓握反射。通过额叶评估量表(FAB)和韦格分类试验(WST)进行认知筛查;同时评估UMN功能障碍。PM、CM和MY的检出率更高(分别占ALS样本的62%、52%和44%),而其他反射的比例较低。表现出三种或更多PRs的患者FAB和WST评分显著更低。另一方面,UMN功能障碍与PRs仅存在中度关联。总之,PRs评估是筛查ALS患者认知障碍的一种有前景的辅助工具;然而,相对于现有的ALS专用认知筛查工具,有必要进一步研究以确定其附加价值。

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