Hartley W J, Canfield P J, Donnelly T M
Acta Neuropathol. 1982;56(3):225-32. doi: 10.1007/BF00690639.
This paper describes three cases of what is probably a new form of storage disorder. The affected animals were English Springer Spaniels and they had developed progressive neurological signs when 2-3 years old. They had gross enlargement of the vagi and of the spinal nerves supplying the brachial plexus. By light microscopy in these nerves these were massive amounts of endoneurial loose fibrous tissue with dispersion of nerve fibres and many foamy phagocyte-like cells. In the central nervous system there was very severe cytoplasmic vacuolation of most neurones and neuroglia, perivascular phagocyte-like cells, loss of myelin, loss of Purkinje cells, numerous spheroids, hypertrophic astrocytes and fibrous astrocytosis. There was also foamy cytoplasmic vacuolation of renal convoluted tubules and pancreatic exocrine parenchyma and numerous foamy phagocyte-like cells in lymph node and lung. On ultrastructural examination membrane bound vacuoles were present in the cytoplasm of affected neurones and phagocyte-like cells. Most of the vacuoles were empty, some contained amorphous materials and some, in neurones, contained stacks of curved or straight lamellae. It is suggested that the stored material could be an oligosaccharide.
本文描述了三例可能是一种新型贮积病的病例。患病动物为英国激飞猎犬，在2至3岁时出现进行性神经症状。迷走神经以及供应臂丛神经的脊神经显著增粗。在这些神经的光镜检查中，可见大量神经内膜疏松纤维组织，神经纤维分散，并有许多泡沫状吞噬细胞样细胞。在中枢神经系统，大多数神经元和神经胶质细胞出现非常严重的细胞质空泡化，血管周围有吞噬细胞样细胞，髓鞘脱失，浦肯野细胞丢失，有大量小球体、肥大星形胶质细胞和纤维性星形细胞增生。肾曲管和胰腺外分泌实质也有泡沫状细胞质空泡化，淋巴结和肺中有大量泡沫状吞噬细胞样细胞。超微结构检查显示，受影响的神经元和吞噬细胞样细胞的细胞质中有膜结合空泡。大多数空泡是空的，有些含有无定形物质，有些在神经元中含有弯曲或直的板层堆叠。提示储存物质可能是一种寡糖。
