Creutzfeldt-Jakob病二例电镜观察

本实验用透射电镜观察了二例Ｃｒｅｕｔｚｆｅｌｄｔ－Ｊａｋｏｂ病（ＣＪＤ）的尸检及活检脑组织。其中一例为死后６天尸检脑组织，电镜下可见神经元及神经胶质细胞明显变性，核染色质块状凝集，核碎裂，可见细胞碎片。另一例为穿刺活检脑组织，因取材过少光镜病理检查只发现脑组织水肿，不能明确诊断，电镜观察二例均少见神经元，神经胶质细胞多见且变性，最突出的表现为脑组织的海绵状界膜空泡变性，有髓神经纤维髓鞘解离并有部分溶解，轴索溶解消失并呈空泡样变性。本实验表明：对临床上表现为进行性痴呆，脑电图广泛异常的病人的组织活检，送检组织过少，一般病理检查不能明确诊断时，电镜检查具有重要意义，即使是死亡６天后的尸检仍可为ＣＪＤ这种疑难和罕见病例的确诊提供有力的证据。

Ultrastructural observation on two cases of Creutzfeldt-Jakob Disease

Two cases of brain biopsy and autopsy tissues of Creutzfeldt Jakob disease(CJD)were observed under electron microscope.One case was brain autopsy tissue that died 6 days ago.Neurons and neuroglia cells obviously degenerated,nucleal chromatin condensed to massed or broke into pieces and cellular pieces could be observed.The other case was brain biopsy tissue,light microscopic pathologic examination could only find the dropsy in brain tissue and could not make the final diagnosis.Fewer neurons could be found under the electron microscope and many glial cells could be found and degenerated.The remarkable ultrastructural change was spongiform membrane bound vacuoles degeneration and myelin sheath of myelinated nerve fibers disintegrated and part of them dissolved,the axons dissolved and showed the vacuoles degeneration.This observation gives the evidence that abnormal electroencephalography(EEG) examination,the brain biopsy specimens of the patients with progressive dementia,and may be too little or too late to make the final diagnosis with light microscopic examination,the electron microscopic examination is of great significance for the ultrastructural diagnosis of CJD.