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肌萎缩侧索硬化症的进展:伊朗肌萎缩侧索硬化症临床登记,一项多中心研究。

Amyotrophic lateral sclerosis progression: Iran-ALS clinical registry, a multicentre study.

作者信息

Shamshiri Hosein, Fatehi Farzad, Davoudi Farnoush, Mir Elham, Pourmirza Behin, Abolfazli Roya, Etemadifar Masoud, Harirchian Mohammad Hossein, Gharagozli Koroush, Ayromlou Hormoz, Basiri Keivan, Zamani Babak, Rohani Mohammad, Sedighi Behnaz, Roudbari Ali, Delavar Kasmaei Hossein, Nikkhah Karim, Ranjbar Naeini Alireza, Nafissi Shahriar

机构信息

a Iranian Center of Neurological Research, Department of Neurology , Tehran University of Medical Sciences , Tehran.

b Department of Community Medicine , Iran University of Medical Sciences , Tehran.

出版信息

Amyotroph Lateral Scler Frontotemporal Degener. 2015;16(7-8):506-11. doi: 10.3109/21678421.2015.1074698. Epub 2015 Oct 5.

Abstract

This study was designed to evaluate ALS progression among different subgroups of Iranian patients. Three hundred and fifty-eight patients from centres around the country were registered and their progression rate was evaluated using several scores including Manual Muscle Test scoring (MMT) and the revised ALS Functional Rating Scale (ALSFRS-R). Progression rate was analysed separately in subgroups regarding gender, onset site, stage of disease and riluzole consumption. A significant difference in MMT deterioration rate (p = 0.01) was noted between those who used riluzole and those who did not. No significant difference was observed in progression rates between male/female and bulbar-onset/limb-onset groups using riluzole. In conclusion, riluzole has a significant effect on muscle force deterioration rate but not functional scale. Progression rate was not influenced by site of onset or gender.

摘要

本研究旨在评估伊朗患者不同亚组中的肌萎缩侧索硬化症(ALS)进展情况。来自该国各地中心的358名患者进行了登记,并使用包括徒手肌力测试评分(MMT)和修订的ALS功能评定量表(ALSFRS-R)在内的多种评分来评估他们的进展率。根据性别、发病部位、疾病阶段和是否使用利鲁唑,在亚组中分别分析进展率。使用利鲁唑和未使用利鲁唑的患者之间,MMT恶化率存在显著差异(p = 0.01)。在使用利鲁唑的男性/女性和延髓起病/肢体起病组之间,进展率未观察到显著差异。总之,利鲁唑对肌力恶化率有显著影响,但对功能量表无显著影响。进展率不受发病部位或性别的影响。

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