Programme National de Nutrition, Kinshasa, Democratic Republic of the Congo.
PLoS Negl Trop Dis. 2012;6(7):e1759. doi: 10.1371/journal.pntd.0001759. Epub 2012 Jul 31.
Konzo (caused by consumption of improperly processed cassava, Manihot esculenta) and neurolathyrism (caused by prolonged overconsumption of grass pea, Lathyrus sativus) are two distinct non-infectious upper motor neurone diseases with identical clinical symptoms of spastic paraparesis of the legs. They affect many thousands of people among the poor in the remote rural areas in the central and southern parts of Africa afflicting them with konzo in Ethiopia and in the Indian sub-continent with neurolathyrism. Both diseases are toxico-nutritional problems due to monotonous consumption of starchy cassava roots or protein-rich grass pea seeds as a staple, especially during drought and famine periods. Both foods contain toxic metabolites (cyanogenic glycosides in cassava and the neuro-excitatory amino acid β-ODAP in grass pea) that are blamed for theses diseases. The etiology is also linked to the deficiency in the essential sulfur amino acids that protect against oxidative stress. The two diseases are not considered reportable by the World Health Organization (WHO) and only estimated numbers can be found. This paper analyzes research performance and determines scientific interest in konzo and neurolathyrism. A literature search of over 21 years (from 1990 to 2010) shows that in terms of scientific publications there is little interest in these neglected motorneurone diseases konzo and neurolathyrism that paralyze the legs. Comparison is made with HTLV-1/TSP, an infectious disease occurring mainly in Latin America of which the clinical manifestation is similar to konzo and neurolathyrism and requires a differential diagnosis. Our findings emphasize the multidisciplinary nature of studies on these neglected diseases, which however have not really captured the attention of decision makers and project planners, especially when compared with the infectious HTLV-1/TSP. Konzo and neurolathyrism can be prevented by a balanced diet.
科酮病（由食用不当加工的木薯引起，Manihot esculenta）和洛夫田诱发性神经病（由长期过量食用兵豆引起，Lathyrus sativus）是两种截然不同的非传染性上运动神经元疾病，具有相同的腿部痉挛性截瘫的临床症状。它们影响到非洲中部和南部偏远农村地区的许多贫困人口，在埃塞俄比亚引起科酮病，在印度次大陆引起洛夫田诱发性神经病。这两种疾病都是由于单调地食用富含淀粉的木薯根或富含蛋白质的兵豆种子作为主食而引起的毒理性营养问题，尤其是在干旱和饥荒时期。这两种食物都含有有毒的代谢物（木薯中的氰苷和兵豆中的神经兴奋型氨基酸β-ODAP），这些代谢物被认为是这些疾病的病因。病因也与必需硫氨基酸的缺乏有关，这种氨基酸可以预防氧化应激。这两种疾病都不被世界卫生组织（WHO）认为是需要报告的疾病，只能找到估计的数字。本文分析了科酮病和洛夫田诱发性神经病的研究表现和确定科学兴趣。对 21 年（1990 年至 2010 年）的文献进行搜索表明，就科学出版物而言，人们对这些被忽视的运动神经元疾病科酮病和洛夫田诱发性神经病（使腿部瘫痪）的兴趣不大。将其与 HTLV-1/TSP 进行了比较，后者是一种主要发生在拉丁美洲的传染病，其临床表现与科酮病和洛夫田诱发性神经病相似，需要进行鉴别诊断。我们的研究结果强调了对这些被忽视疾病进行多学科研究的性质，但这些疾病并没有真正引起决策者和项目规划者的注意，特别是与传染性 HTLV-1/TSP 相比时更是如此。科酮病和洛夫田诱发性神经病可以通过均衡饮食来预防。
