Creutzfeldt—Jakob病的临床及病理分析

目的 探讨Creutzfeldt-Jakob病（CJD）的临床特点及诊断,提高生前确诊率。方法 对4例经病理证实的CJD患者的临床表现、光镜及电镜所见进行分析。结果 4例均具有典型的临床表现,病理可见神经细胞变性死亡,胶质细胞增生,无炎性改变,电镜除可见上述改变外,还可见部分髓鞘及轴索的肿胀、变性。结论 此病多为散发性,无特殊有效治疗,预后差。临床病理检查是确诊CJD的最佳检查方法。

Clinical and pathological analysis of Creutzfeldt-Jakob disease

Objective To study clinical characters and diagnosis of Creutzfeldt Jakob disease (CJD), which will raise the rate of confirmed diagnosis.Methods The clinical manifestation, the results of light microscope and electron microscope in 4 patients with CJD proved by pathological examination were analyzed.Results There was typical clinical manifestation in the 4 patients,neuron degenerative death,gliocyte hyperplasia,no inflammatory change were observed in pathological examination. Degeneration and oncotic of axon in some myelin sheath were found by electron microscope examination.Conclusion CJD is a sporadic desease,no special effective therapy and worse prognosis. Clinical pathological examination is the best method for the diagnosis of CJD.