西安交通大学学报（医学版）Issue(4)：505-508,542,5.DOI:10.7652/jdyxb201504016
散发性肌萎缩侧索硬化症起病部位和进展模式及其相关影响因素分析
Disease onset regions and spreading patterns in sporadic amyotrophic lateral sclerosis and related influencing factors
摘要
Abstract
Objective To evaluate the disease onset regions and spreading patterns in sporadic amyotrophic lateral sclerosis (ALS)patients and related influencing factors.Methods We performed a prospective analysis of 1 58 ALS patients.The disease-onset was confirmed according to the patients’self-reports,neurological examination results and electromyogram study.We followed up 1 5 1 patients with the second or other affected body regions during the disease progression.Data were analyzed according to the different groups of onset regions.Results 1.In 94.3% (149/1 58)of the patients,the early motor manifestations were focally in the bulbar,upper or lower limbs.2.The region of onset was associated with signs of lower motor neuron (LMN)and upper motor neuron (UMN)involvement (P = 0.000 ).The LMN involvement was more distinctive in patients with bulbar onset (65.4%,1 7/26 )group.Patients with cervical onset more frequently showed pure LMN (47.9%,45/94 )or concomitant UMN (52.1%,49/94)signs in the affected limbs.The highest proportion of UMN and LMN signs in the affected lower limb was found in the lumbar onset (83.8%,31/37 )group.3.Spreading patterns:Rostral to caudal spreading pattern was more frequent in bulbar onset patients.For patients with limb onset,there were significant differences between spreading patterns and disease-onset regions (P =0.04).Circular (31.5%,28/89),horizontal (30.3%,31/89)and vertical (21.3%,1 9/89)spreading patterns were more frequent in cervical onset patients whereas circular (47.2%,1 7/36)spreading patterns were more frequent in lumbar onset patients.4.There was a strong association between the rate of progression and age of disease onset (P =0.01 1).Patients aged over 60 had a faster progression.Conclusion ALS is a focal process at motor axis along the spinal cord and cerebral cortex.Different disease-onset can cause different distribution of UMN and LMN signs.Therefore,special attention should be paid to the signs of disease-onset clinically.ALS does start focally and spreads to adjacent regions.Elder patients have a faster disease progression.关键词
肌萎缩侧索硬化/上运动神经元/下运动神经元/起病部位/进展类型Key words
amyotrophic lateral sclerosis/upper motor neuron/lower motor neuron/region of disease onset/spreading pattern分类
医药卫生引用本文复制引用
党静霞,靳娇婷,胡芳芳,贾蕊..散发性肌萎缩侧索硬化症起病部位和进展模式及其相关影响因素分析[J].西安交通大学学报（医学版）,2015,(4):505-508,542,5.基金项目
陕西省科技攻关项目(No.2014K11-02-01-04) Supported by the Social Development Research Project of Shaanxi Province(No.2014K11-02-01-04) （No.2014K11-02-01-04）
