Ludolph A C, Spencer P S
Department of Neurology, Humboldt University, Berlin, Germany.
J Neurol Sci. 1996 Aug;139 Suppl:53-9. doi: 10.1016/0022-510x(96)00122-0.
Although neurotoxic models for progressive degeneration of both the anterior horn cell and the Betz cell do not exist, (neuro)lathyrism and neurocassavism (konzo) are examples of self limiting neurotoxic disorders that predominantly target the Betz cell. Both disorders are caused by the continuous intake of neurotoxic plant products (Lathyrus sativus and Manihot esculenta, respectively) which result in a virtually identical clinical picture of spastic paraparesis. A neurotoxic excitatory amino acid and AMPA agonist (beta-N-oxalylamino-1-alanine, BOAA) is held largely responsible for lathyrism. Epidemics of konzo are strongly associated with increased intake of the cyanide-liberating glycoside linamarin by protein-poor subjects. Whereas an animal model for neurocassavism does not exist, macaques fed Lathyrus sativus or BOAA develop central motor deficits with corticospinal tract involvement. Estimated dosages of Lathyrus sativus used to induce beginning lathyrism in well-nourished primates are 10-20 fold greater than those associated with irreversible human neurolathyrism. Major unexplained aspects of both diseases are the factors which allow the suspected toxins to target Betsz cells (blood-brain barrier, receptor distribution, cellular energy metabolism), the latency to onset in both diseases, and how two separate etiologic factors trigger clinically similar disorders.
虽然目前尚不存在用于前角细胞和贝茨细胞进行性退化的神经毒性模型，但（神经）山黧豆中毒和神经木薯中毒（konzo）是主要针对贝茨细胞的自限性神经毒性疾病的例子。这两种疾病都是由于持续摄入神经毒性植物产品（分别是山黧豆和木薯）所致，它们会导致几乎相同的痉挛性截瘫临床症状。一种神经毒性兴奋性氨基酸和AMPA激动剂（β-N-草酰氨基-L-丙氨酸，BOAA）在很大程度上是山黧豆中毒的病因。konzo的流行与蛋白质缺乏人群摄入释放氰化物的糖苷亚麻苦苷增加密切相关。虽然不存在神经木薯中毒的动物模型，但喂食山黧豆或BOAA的猕猴会出现涉及皮质脊髓束的中枢运动功能缺陷。在营养良好的灵长类动物中，用于诱发初期山黧豆中毒的山黧豆估计剂量比与人类不可逆神经山黧豆中毒相关的剂量大10至20倍。这两种疾病主要的未解之谜包括使疑似毒素靶向贝茨细胞的因素（血脑屏障、受体分布、细胞能量代谢）、两种疾病发病的潜伏期，以及两种不同的病因如何引发临床症状相似的疾病。
