异常PrP沉积对人朊蛋白病的临床和病理影响

Effect of deposition of abnormal prion protein on clinical and pathological aspects of human prion disease

目的 探讨异常ＰｒＰ沉积形式与ＣｒｅｕｔｚｆｅｌｄｔＪａｋｏｂ 病（ＣＪＤ）、ＧｅｒｓｔｍａｎｎＳｔｒａｕｓｓｌｅｒ 综合征（ＧＳＳ）临床与病理关系。方法 经病理检查确诊的７ 例ＣＪＤ，１ 例ＧＳＳ脑切片进行朊蛋白ＰｒＰ免疫组化染色和实验动物传递。结果 （１） ７ 例ＣＪＤ均呈现突触型阳性，而ＧＳＳ则呈现斑块型阳性；（２） 突触型阳性者，病程短，平均１２ ．３ 个月，斑块型阳性者病程６０ 个月；（３） ７ 例突触型均有痴呆，６ 例有肌阵挛，小脑症状不明显，斑块型则与其相反；（４） 突触型中４ 例进行动物传递均获成功，斑块型则否；（５） 突触型ＰｒＰ沉积多位于大脑灰质，斑块型多在小脑分子层。结论 应用水解高压灭菌法，以ＰｒＰ抗血清为第一抗体的免疫组化方法，可以准确判定异常ＰｒＰ沉积的两种类型，二者神经症状、病程长短、脑电图改变以及动物传递结果等均不相同。

Objective To investigate the relationship of deposited type of abnormal prion protein with clinical pictures and neuropathological changes in Creutzfeldt Jakob disease(CJD) and Gerstmann Straussler Scheinker syndrome(GSS). Methods Immunohistochemicol staining with an antiserum to prion protein (PrP) using hydrolytic autoclaving pretreatment and animal transmission were carried out in seven cases with CJD and one case with GSS. Results (1) Positive staining with the synaptic type was seen in all of the seven cases with CJD, with plaque type in one case with GSS. (2)The average ,short illness duration, was 12.6 months, in CJD, but the illness duration in the case of GSS was sixty months. (3)The myoclonus and dementia were signifcantly seen in CJD,but the cerebellar symptom was obvious in GSS. (4)Successful animal transmission could be determined in CJD. (5) Deposition of abnormal prion protein in the synaptic type mainly located in the grey matter of the cerebrum,in the plague type located in the molecular layer of the cerebellum. Conclusion The two types of deposition of abnormal prion protein can be determined with immunostaining. Significant differences for the plaque type were found in the synaptic type in regard with the illness duration, neurological symptoms, EEG changes and animal transmission.