Kim R C, Collins G H, Parisi J E, Wright A W, Chu Y B
Brain. 1981 Mar;104(Pt 1):61-78. doi: 10.1093/brain/104.1.61.
A family is described in which 4 of 10 siblings developed a dementing illness that culminated in death within five to six years of onset. The pathological findings in 3 members were strikingly similar, and consisted of widespread nerve cell loss and astrocytosis within the cerebral cortex, status spongiosus within the outer cortical layers and, in 2, nerve cell loss and astrocytosis within the dorsomedial nucleus of the thalamus. It is concluded that the disorder described in this report does not conform precisely to any of the currently recognized categories of familial dementing disease.
本文描述了一个家庭，该家庭中10个兄弟姐妹中有4人患上了一种导致痴呆的疾病，发病后五到六年内最终死亡。3名成员的病理检查结果极为相似，包括大脑皮层广泛的神经细胞丢失和星形细胞增多、皮质外层的海绵状状态，以及2例丘脑背内侧核的神经细胞丢失和星形细胞增多。结论是，本报告中描述的疾病并不完全符合目前公认的任何一种家族性痴呆疾病类别。
