目的 探讨皮质?纹状体?脊髓变性( C J D) 的临床特点及诊断.方法 分析经病理证实的5 例 C J D 的临床表现、光镜及超微检查结果.结果 5 例均有典型 C J D 的临床表现,病理检查见神经细胞变性死亡,神经胶质细胞增生,无炎症性改变.超微检查除见上述改变外,还可见神经毡中膜性空泡内有卷曲的膜性碎片,部分髓鞘的轴突肿胀、空化.结论 该文5 例 C J D 除引起中枢神经细胞胞体损害外,还可损害轴突,此病多为散发性,预后极差,目前无特效治疗.“,”Objective To study clinical characters and diagnosis of Creutzfeldt Jakob disease (CJD). Methods The clinical manifestation, results of microscope and ultrastruture in five patients with CJD, which were proved by pathological examination, were analyzed. Results There were typical clinical manifestatioin in five patients. Pathologically, neurone degenerative death, gliocyte hyperplasia and no change of inflammation were observed. Curled membranous fragment in neuropil, and vacuolar and oncotic of axon in some myelin sheath were found by ultrastrucural examination. Conclusions CJD is a unfavourable prognosis, untreated and sporadic disease. The results suggest that CJD not only damage the body but also damage the axon of central neuron.