CAJ | 학술논문

目的：探讨散发性肌萎缩侧索硬化症(ALS)患者首发起病部位和起病后疾病进展模式及其相关影响因素。方法前瞻性设计并对158例 ALS 患者以其主诉和通过神经系统查体和神经电生理检查,确定其首发起病部位,并对其中151例已经出现第二个及之后损害部位的患者进行随访,按照延髓、颈髓及腰髓不同起病部位分组进行相关影响因素研究。结果①首发起病部位局限在延髓、单侧上肢或下肢者149例(94.3%)。②起病部位处上、下运动神经元损害体征与起病部位不同有关,P =0.000,延髓起病者多表现为下运动神经元损害体征(65.4%,17/26)；颈髓起病者多以一侧上肢的单纯下运动神经元损害体征(47.9%,45/94)或合并上运动神经元损害体征(52.1%,49/94)为主；而腰髓起病者主要以一侧下肢上、下运动神经元混合损害体征(83.8%,31/37)为主。③从进展模式上看,延髓起病者以嘴端-尾端垂直型进展多见；肢体起病者,进展类型和起病部位相关,P =0.04,颈髓起病者以循环型(31.5%,28/89)、水平型(30.3%,31/89)和垂直型(21.3%,19/89)进展多见；而腰髓起病者以循环型(47.2%,17/36)进展较多见。④起病后疾病进展率和起病年龄有关,P =0.011,60岁以上起病者进展较快。结论ALS 首发起病部位是在脊髓和大脑皮层运动轴的某个局限部位,首发起病部位不同,上、下运动神经元损害体征的分布及程度也不同,临床上应该重视首发起病部位处的体征。ALS 发病后主要是沿着相邻体区进展,年龄越大,进展越快。
목적：탐토산발성기위축측색경화증(ALS)환자수발기병부위화기병후질병진전모식급기상관영향인소。방법전첨성설계병대158례 ALS 환자이기주소화통과신경계통사체화신경전생리검사,학정기수발기병부위,병대기중151례이경출현제이개급지후손해부위적환자진행수방,안조연수、경수급요수불동기병부위분조진행상관영향인소연구。결과①수발기병부위국한재연수、단측상지혹하지자149례(94.3%)。②기병부위처상、하운동신경원손해체정여기병부위불동유관,P =0.000,연수기병자다표현위하운동신경원손해체정(65.4%,17/26)；경수기병자다이일측상지적단순하운동신경원손해체정(47.9%,45/94)혹합병상운동신경원손해체정(52.1%,49/94)위주；이요수기병자주요이일측하지상、하운동신경원혼합손해체정(83.8%,31/37)위주。③종진전모식상간,연수기병자이취단-미단수직형진전다견；지체기병자,진전류형화기병부위상관,P =0.04,경수기병자이순배형(31.5%,28/89)、수평형(30.3%,31/89)화수직형(21.3%,19/89)진전다견；이요수기병자이순배형(47.2%,17/36)진전교다견。④기병후질병진전솔화기병년령유관,P =0.011,60세이상기병자진전교쾌。결론ALS 수발기병부위시재척수화대뇌피층운동축적모개국한부위,수발기병부위불동,상、하운동신경원손해체정적분포급정도야불동,림상상응해중시수발기병부위처적체정。ALS 발병후주요시연착상린체구진전,년령월대,진전월쾌。
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.