血栓性血小板减少性紫癜的临床神经精神表现和病理改变

报道2例血栓性血小板减少性紫癜的神经精神表现及其中1例的脑病理结果，分析其临床与病理联系。2例病人均为青年女性，有血栓性血小板减少性紫癜的典型临床表现和实验室证据，其神经系统主要表现有语言表达困难，尖叫，谵语，偏瘫以及意识障碍，都表现为急进性过程。例1仅给予激素等治疗，于发病4天后死亡，另1例及时给予血浆置换加激素治疗，临床症状和和实验室指标均恢复正常。例1的脑病理改变为大脑，脑干，小脑广泛分布的中小血管透明样血栓形成，海马，颞叶等皮层神经元严重的缺血缺氧性改变。研究表明，血栓性血小板减少性紫癜的神经系统表现形式多种多样，其病理基础为广泛的微血管病变导致灌注不良，缺血缺氧而非脑实质出血。

CLINICAL FEATURES AND PATHOLOGY OF THE CENTRAL NERVOUS SYSTEM IN THROMBOTIC THROMBOCYTOPENIC PURPURA

To have a better understandings of the clinical features and pathology of the central nervous system of thrombotic thromocytopenic purpura(TTP) in order to avoid its misdiagnosis and improper treatment, we reported here the clinical features of the central nervous system of 2 cases with TTP and pathologic changes in the brain of one case of them.The result showed that in 2 young women presented with atypical clinical features and laboratory findings of TTP, the main presentations of the central nervous system included language disturbances, hemiplegia, delirium, scream, confusion, unconsciousness.Both of them had an acute progresive course. One case died after steroids therapy alone and the other got complete remissions after conbined treatment with plasmapheresis and steroids. Pathological examination of the brain of the died case showed widespread hyaline thrombosis in small vessels and ischaemic changes of the neurons in the hippocampus and the cerebral cortex. Our conclusion is there are various patterns of clinical presentations of the CNS in TTP and the disturbances are due to widespread thrombotic microangiopathy and failure of cerebral perfusion.