Konzo is a toxico-nutritional upper motor neuron disease causing a spastic paraparesis in schoolchildren and childbearing women in some African countries. Almost a century since the first description of konzo, its underlying etiopathogenic mechanisms and causative agent remain unknown. This paper aims at refreshing the current knowledge of konzo determinants and pathogenesis in order to enlighten potential new research and management perspectives. Literature research was performed in PubMed and Web of Science databases according to the PRISMA methodology. Available data show that cassava-derived cyanide poisoning and protein malnutrition constitute two well-documented risk factors of konzo. However, observational studies have failed to demonstrate the causal relationship between konzo and cyanide poisoning. Thiocyanate, the current marker of choice of cyanide exposure, may underestimate the actual level of cyanide poisoning in konzo patients as a larger amount of cyanide is detoxified via other unusual pathways in the context of protein malnutrition characterizing these patients. Furthermore, the appearance of konzo may be the consequence of the interplay of several factors including cyanide metabolites, nutritional deficiencies, psycho-emotional and geo-environmental factors, resulting in pathophysiologic phenomena such as excitotoxicity or oxidative stress, responsible for neuronal damage that takes place at sparse cellular and/or subcellular levels.

Konzo 是一种有毒营养性上运动神经元疾病，在一些非洲国家会导致学童和育龄妇女出现痉挛性截瘫。自第一次描述 konzo 以来近一个世纪，其潜在的致病机制和病原体仍然未知。本文旨在更新当前关于 konzo 决定因素和发病机制的知识，以启发潜在的新研究和管理观点。根据 PRISMA 方法，在 PubMed 和 Web of Science 数据库中进行了文献研究。现有数据表明，木薯来源的氰化物中毒和蛋白质营养不良构成了两个有据可查的 konzo 风险因素。然而，观察性研究未能证明 konzo 和氰化物中毒之间的因果关系。硫氰酸盐，目前选择氰化物暴露的标志物可能低估了 konzo 患者氰化物中毒的实际水平，因为在这些患者的蛋白质营养不良特征的背景下，大量的氰化物通过其他不寻常的途径解毒。此外，konzo 的出现可能是多种因素相互作用的结果，包括氰化物代谢物、营养缺乏、心理情绪和地理环境因素，导致兴奋性毒性或氧化应激等病理生理现象，导致发生的神经元损伤在稀疏的细胞和/或亚细胞水平。可能低估了 konzo 患者氰化物中毒的实际水平，因为在这些患者的蛋白质营养不良的背景下，大量的氰化物通过其他不寻常的途径解毒。此外，konzo 的出现可能是多种因素相互作用的结果，包括氰化物代谢物、营养缺乏、心理情绪和地理环境因素，导致兴奋性毒性或氧化应激等病理生理现象，导致发生的神经元损伤在稀疏的细胞和/或亚细胞水平。可能低估了 konzo 患者氰化物中毒的实际水平，因为在这些患者的蛋白质营养不良的背景下，大量的氰化物通过其他不寻常的途径解毒。此外，konzo 的出现可能是多种因素相互作用的结果，包括氰化物代谢物、营养缺乏、心理情绪和地理环境因素，导致兴奋性毒性或氧化应激等病理生理现象，导致发生的神经元损伤在稀疏的细胞和/或亚细胞水平。