Jamroz E, Marszał E, Glinka Z, Fidziańska A
Department of Neuropediatrics of Silesian School of Medicine, Katowice.
Folia Neuropathol. 1994;32(2):81-6.
The ultrastructure of peripheral blood lymphocytes of 30 children with degenerative central nervous system diseases was analyzed. The affected children were divided into four groups. Lysosomal storage were characterized by the storage of membrane-bound inclusions in peripheral lymphocytes. Ceroidlipofuscinosis was manifested by the presence of curvilinear bodies. The appearance of abnormal mitochondria was found in mitochondrial encephalopathies. The tubuloreticular structures seen in lymphocytes of some unclassified cases suggested viral factors that acted in prenatal life. All findings confirm the role of peripheral lymphocytes analysis in the diagnosis of degenerative central nervous system diseases.
对30例中枢神经系统退行性疾病患儿的外周血淋巴细胞超微结构进行了分析。患病儿童被分为四组。溶酶体贮积症的特征是外周淋巴细胞中存在膜结合包涵体。蜡样脂褐质沉积症表现为曲线体的存在。线粒体脑肌病中发现了异常线粒体。在一些未分类病例的淋巴细胞中看到的管状网状结构提示了产前起作用的病毒因素。所有发现均证实外周淋巴细胞分析在中枢神经系统退行性疾病诊断中的作用。