Patterns of symptom development in patients with motor neuron disease
Patterns of symptom development in patients with motor neuron disease
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DOI：
10.1080/21678421.2017.1386688
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发表时间：
2018-01-01
影响因子：
2.8
通讯作者：
Van Den Berg, Leonard H.
中科院分区：
文献类型：
--
作者：
Walhout, Renee;Verstraete, Esther;Van Den Berg, Leonard H.
目的：探讨运动神经元病（MND）的症状发展是一个随机的还是有组织的过程。研究方法：对600例肌萎缩侧索硬化（ALS）、上运动神经元（UMN）或下运动神经元（LMN）表型患者进行了症状发展问卷调查。使用二项检验检查症状从发病部位的分布。通过Kaplan-Meier分析评价症状随时间的发展。结果：共有470名受访者（ALS = 254; LMN = 100; UMN = 116）。随后的症状更常见于单侧肢体发病后的对侧肢体（ALS：臂p = 1.05 x 10（-8），腿p < 2.86 x 10（-15）; LMN表型：臂p = 6.74 x 10（-9），腿p = 6.26 x 10（-6）; UMN表型：腿p = 4.07 x 10（-14））。在肢体发病的患者中，对侧肢体的症状发生明显更快，其次是其他肢体，最后是延髓区。还报告了非连续症状发展模式：30%的ALS患者在延髓发作后出现腿部症状，11%的ALS患者在腿部发作后出现延髓症状。结论：症状从一侧肢体向对侧肢体和邻近部位的优选传播似乎是MND表型的特征，表明症状传播是有组织的，可能涉及轴突连接。然而，非连续性症状发展并不罕见，可能涉及其他因素。
Objective: To investigate whether symptom development in motor neuron disease (MND) is a random or organized process. Methods: Six hundred patients with amyotrophic lateral sclerosis (ALS), upper motor neuron (UMN) or lower motor neuron (LMN) phenotypes were invited for a questionnaire concerning symptom development. A binomial test was used to examine distribution of symptoms from site of onset. Development of symptoms over time was evaluated by Kaplan-Meier analysis. Results: There were 470 respondents (ALS = 254; LMN = 100; UMN = 116). Subsequent symptoms were more often in the contralateral limb following unilateral limb onset (ALS: arms p = 1.05 x 10(-8), legs p < 2.86 x 10(-15); LMN phenotype: arms p = 6.74 x 10(-9), legs p = 6.26 x 10(-6); UMN phenotype: legs p = 4.07 x 10(-14)). In patients with limb onset, symptoms occurred significantly faster in the contralateral limb, followed by the other limbs and lastly by the bulbar region. Patterns of non-contiguous symptom development were also reported: leg symptoms followed bulbar onset in 30%, and bulbar symptoms followed leg onset in 11% of ALS patients. Conclusions: Preferred spread of symptoms from one limb to the contralateral limb, and to adjacent sites appears to be a characteristic of MND phenotypes, suggesting that symptom spread is organized, possibly involving axonal connectivity. Non-contiguous symptom development, however, is not uncommon, and may involve other factors.