Vuia O
J Neurol Sci. 1978 Nov;39(1):37-46. doi: 10.1016/0022-510x(78)90186-7.
A case is reported of progressive dementia and a terminal picture of generalized tetaniform contractures. The relationship of the generalized tetaniform contractures to the stiff-man syndrome is discussed. Morphologically, diffuse amyloid deposition was found in the pial and cortical vessels, accompanied by amyloid deposition in the senile plaques in the cortical and cerebellar cortex. Apart from the typical staining and ultrastructural aspects of amyloid, a deposition of material was observed, corresponding in optical and electron microscopy to a paraprotein. This case demonstrated not only the relationship between the deposition of amyloid and the formation of senile plaques, but also sustains the direct connection between amyloid in senile plaques and the paraprotein substances deriving from the blood. The probable relationship between the unusual deposition of paraproteins in the vessels and nervous system and the treatment with immunoglobulins is discussed.
报告了一例进行性痴呆及终末期全身性破伤风样挛缩的病例。讨论了全身性破伤风样挛缩与僵人综合征的关系。形态学上，在软脑膜和皮质血管中发现弥漫性淀粉样沉积，同时在皮质和小脑皮质的老年斑中也有淀粉样沉积。除了淀粉样物质典型的染色和超微结构特征外，还观察到一种物质沉积，在光学显微镜和电子显微镜下与副蛋白相符。该病例不仅证明了淀粉样沉积与老年斑形成之间的关系，还支持了老年斑中的淀粉样物质与源自血液的副蛋白物质之间的直接联系。讨论了副蛋白在血管和神经系统中异常沉积与免疫球蛋白治疗之间可能的关系。
