Marin Raul E, Micheloud Juan F, Vignale Nilda D, Gimeno Eduardo J, O'Toole Donal, Gardner Dale R, Woods Leslie, Uzal Francisco A
Facultad Ciencias Agrarias, Universidad Nacional de Jujuy, Jujuy, Argentina (Marin, Vignale).
Área de Sanidad Animal, Instituto Nacional de Tecnología Agropecuaria, Salta, Argentina (Micheloud).
J Vet Diagn Invest. 2020 May;32(3):467-470. doi: 10.1177/1040638720914338. Epub 2020 Apr 1.
Lysosomal storage diseases are inherited and acquired disorders characterized by dysfunctional lysosomes. Intracytoplasmic accumulation of undegraded substrates leads to impaired cellular function and death. Several plant species are toxic to livestock because of the presence of indolizidine alkaloids, including swainsonine, which cause a storage disease. Swainsonine-induced nervous disease (i.e., locoism) of sheep and cattle is well recognized in several parts of the world, particularly in the western United States and in parts of Australia. Spontaneous intoxication by var. was suspected in a group of 70 llamas () in Jujuy Province, northwestern Argentina. The animals grazed an area dominated by stands of var. . Clinical signs were staggering, ataxia, hypermetria, and progressive weight loss. The clinical course in individual animals was ~50 d. The main microscopic changes were Purkinje cell degeneration, necrosis, and loss, associated with intracytoplasmic vacuolation, meganeurite formation, and Wallerian degeneration. Specific positive labeling for ubiquitin was observed in axonal spheroids. Composite leaf and stem samples of var. analyzed by high-performance liquid chromatography contained 0.03% swainsonine. Based on the microscopic lesions, clinical history, and plant analysis, a diagnosis was made of storage disease caused by consumption of swainsonine-containing var. .
溶酶体贮积病是一类遗传性和获得性疾病,其特征为溶酶体功能障碍。未降解底物在细胞质内蓄积导致细胞功能受损和死亡。几种植物因含有吲哚里西啶生物碱(包括苦马豆素)而对家畜有毒,可引发一种贮积病。绵羊和牛的苦马豆素诱发的神经疾病(即疯草病)在世界多个地区都广为人知,尤其是在美国西部和澳大利亚部分地区。在阿根廷西北部胡胡伊省的一群70只美洲驼中怀疑发生了由 变种引起的自发中毒。这些动物在一片以 变种为主的区域放牧。临床症状为蹒跚、共济失调、运动过度和体重逐渐减轻。个体动物的病程约为50天。主要的微观变化为浦肯野细胞变性、坏死和丢失,伴有细胞质空泡化、巨神经纤维形成和华勒氏变性。在轴突球体中观察到泛素特异性阳性标记。通过高效液相色谱分析的 变种复合叶和茎样本含有0.03%的苦马豆素。基于微观病变、临床病史和植物分析,诊断为由食用含苦马豆素的 变种引起的贮积病。