PDF(Pubmed)
Abstract：
BACKGROUND: Neuroanatomical staging of sporadic amyotrophic lateral sclerosis (ALS) indicates that neurodegeneration may spread corticofugally.
METHODS: We conducted an observational study to define the initial sites of disease onset and the clinical progression (\'spreading patterns\') of motor deficits in a cohort of 910 ALS patients in Germany.
RESULTS: Mean age of ALS onset was 59.0 ± 12.6 years for males and 61.2 ± 10.5 years for females, the mean ALSFRS-R was 35.1 ± 9.2, and 7.7% of the cohort reported a family history. Onset of motor symptoms was bulbar/upper limb in 26.8%/35.9%, the right arm initially being slightly more often affected than the left (18.5% vs.16.3%). Testing on concordance of handedness and onset in the dominant arm did not reach significance. Lower limb onset was observed in 37.3%. Unilateral limb onset patients reported horizontal spreading about three times more often than vertical spreading. 71/244 bulbar onset patients reported spreading pattern to the legs, and 17/339 lumbar onset patients reported spreading secondarily to the bulbar region.
CONCLUSIONS: Our results indicate that, although the phenotype of so-called \'spinal\' or \'intraspinal\' spreading predominated, we also observed an additional clinical spreading pattern: 29.1% of patients with bulbar onset experienced spreading clinically to the legs (vice versa in 5.0% of lumbar onset patients). For obvious neuroanatomical reasons, this pattern hardly can be explained solely by a \'spinal\' or an \'intraspinal\' pattern of spreading. Instead, these findings complement insights from previous clinical and clinicopathological studies supporting a cortical initiation of ALS.
METHODS: We conducted an observational study to define the initial sites of disease onset and the clinical progression (\'spreading patterns\') of motor deficits in a cohort of 910 ALS patients in Germany.
RESULTS: Mean age of ALS onset was 59.0 ± 12.6 years for males and 61.2 ± 10.5 years for females, the mean ALSFRS-R was 35.1 ± 9.2, and 7.7% of the cohort reported a family history. Onset of motor symptoms was bulbar/upper limb in 26.8%/35.9%, the right arm initially being slightly more often affected than the left (18.5% vs.16.3%). Testing on concordance of handedness and onset in the dominant arm did not reach significance. Lower limb onset was observed in 37.3%. Unilateral limb onset patients reported horizontal spreading about three times more often than vertical spreading. 71/244 bulbar onset patients reported spreading pattern to the legs, and 17/339 lumbar onset patients reported spreading secondarily to the bulbar region.
CONCLUSIONS: Our results indicate that, although the phenotype of so-called \'spinal\' or \'intraspinal\' spreading predominated, we also observed an additional clinical spreading pattern: 29.1% of patients with bulbar onset experienced spreading clinically to the legs (vice versa in 5.0% of lumbar onset patients). For obvious neuroanatomical reasons, this pattern hardly can be explained solely by a \'spinal\' or an \'intraspinal\' pattern of spreading. Instead, these findings complement insights from previous clinical and clinicopathological studies supporting a cortical initiation of ALS.
摘要：
背景：散发性肌萎缩侧索硬化症（ALS）的神经解剖学分期表明神经变性可能在皮质营养上扩散。
方法：我们在德国910名ALS患者队列中进行了一项观察性研究，以确定疾病发作的初始部位和运动缺陷的临床进展（“传播模式”）。
结果：ALS发病的平均年龄男性为59.0±12.6岁，女性为61.2±10.5岁，ALSFRS-R的平均值为35.1±9.2,7.7%的队列报告有家族史.运动症状以延髓/上肢为主，占26.8%/35.9%，右臂最初比左臂受影响的频率略高(18.5%vs.16.3%)。对惯用手和优势臂发作的一致性的测试没有达到显著性。下肢起病占37.3%。单侧肢体发作患者报告的水平传播频率是垂直传播的三倍。71/244球发病患者报告腿部扩散模式，17/339例腰椎起病患者报告继发扩散至球区域。
结论：我们的结果表明，尽管所谓的“脊柱”或“脊柱内”扩散的表型占主导地位，我们还观察到另外一种临床扩散模式:29.1%的球起病患者经历了临床向腿部的扩散(5.0%的腰起病患者也是如此).出于明显的神经解剖学原因，这种模式很难仅仅用“脊柱”或“脊柱内”扩散模式来解释。相反，这些发现补充了先前支持ALS皮质起始的临床和临床病理研究的见解.
方法：我们在德国910名ALS患者队列中进行了一项观察性研究，以确定疾病发作的初始部位和运动缺陷的临床进展（“传播模式”）。
结果：ALS发病的平均年龄男性为59.0±12.6岁，女性为61.2±10.5岁，ALSFRS-R的平均值为35.1±9.2,7.7%的队列报告有家族史.运动症状以延髓/上肢为主，占26.8%/35.9%，右臂最初比左臂受影响的频率略高(18.5%vs.16.3%)。对惯用手和优势臂发作的一致性的测试没有达到显著性。下肢起病占37.3%。单侧肢体发作患者报告的水平传播频率是垂直传播的三倍。71/244球发病患者报告腿部扩散模式，17/339例腰椎起病患者报告继发扩散至球区域。
结论：我们的结果表明，尽管所谓的“脊柱”或“脊柱内”扩散的表型占主导地位，我们还观察到另外一种临床扩散模式:29.1%的球起病患者经历了临床向腿部的扩散(5.0%的腰起病患者也是如此).出于明显的神经解剖学原因，这种模式很难仅仅用“脊柱”或“脊柱内”扩散模式来解释。相反，这些发现补充了先前支持ALS皮质起始的临床和临床病理研究的见解.
