Kihira Tameko, Yoshida Sohei, Kondo Tomoyoshi, Yase Yoshiro, Ono Seiitsu
Department of Neurology, Wakayama Medical University, 811-1 Kimiidera, Wakayama City 641-8510, Japan.
J Neurol Sci. 2004 Apr 15;219(1-2):7-14. doi: 10.1016/j.jns.2003.11.010.
Epidemiologic studies of endemic foci of amyotrophic lateral sclerosis (ALS) have shown low concentrations of Ca/Mg and high concentrations of Al/Mn in the drinking water and garden soil, which may play a causative role in the pathogenesis of endemic ALS. We studied the effects of chronic exposure to a low-Ca/Mg high-Al maltol diet on the skin of experimental animals. In ALS patients, atrophy of the epidermis, edematous changes with separated collagen fibrils and an accumulation of amorphous materials between collagen bundles were regarded as pathognomonic skin changes of ALS. Mice chronically fed a low-Ca/Mg high-Al maltol diet showed neuronal degeneration and loss in the spinal cords and cerebral cortices, as well as skin changes including atrophy, separation of collagen fibrils and accumulation of amorphous materials, similar to the skin changes characteristic of ALS. This is the first report of skin changes in animal models similar to those of ALS. We speculate that environmental factors such as chronic low-Ca/Mg high-Al condition play some causative role in the pathogenesis of Kii-ALS.
肌萎缩侧索硬化症（ALS）地方性病灶的流行病学研究表明，饮用水和花园土壤中钙/镁浓度低，铝/锰浓度高，这可能在地方性ALS的发病机制中起致病作用。我们研究了长期暴露于低钙/镁高铝麦芽糖醇饮食对实验动物皮肤的影响。在ALS患者中，表皮萎缩、胶原纤维分离的水肿变化以及胶原束之间无定形物质的积累被视为ALS的特征性皮肤变化。长期喂食低钙/镁高铝麦芽糖醇饮食的小鼠表现出脊髓和大脑皮质中的神经元变性和丧失，以及包括萎缩、胶原纤维分离和无定形物质积累在内的皮肤变化，类似于ALS的特征性皮肤变化。这是关于动物模型中出现类似于ALS皮肤变化的首次报道。我们推测，慢性低钙/镁高铝等环境因素在纪伊型ALS的发病机制中起一定的致病作用。
