Druschky A, Erbguth F, Strauss R, Helm G, Heckmann J, Neundörfer B
Department of Neurology and Internal Medicine I, University of Erlangen-Nuremberg, Erlangen, Germany.
Eur Neurol. 1998 Nov;40(4):220-4. doi: 10.1159/000007983.
Thrombotic thrombocytopenic purpura (TTP) is an uncommon multisystem disease characterized by microangiopathic hemolytic anemia, thrombocytopenic purpura, fluctuating neurologic dysfunctions, renal disease, and fever. Delayed diagnosis and treatment may lead to a high mortality rate that can be as great as 90%. We describe 7 patients in whom CNS involvement was the first clinical manifestation of TTP. Despite early diagnosis and treatment, 3 patients died because of multiple system organ failure. Four patients recovered completely. In patients with focal neurologic disorders in whom infectious CNS diseases were excluded TTP should be considered in the differential diagnosis.
血栓性血小板减少性紫癜（TTP）是一种罕见的多系统疾病，其特征为微血管病性溶血性贫血、血小板减少性紫癜、波动性神经功能障碍、肾脏疾病和发热。诊断和治疗延迟可能导致高达90%的高死亡率。我们描述了7例以中枢神经系统受累为TTP首发临床表现的患者。尽管早期诊断并进行了治疗，但仍有3例患者因多系统器官衰竭死亡。4例患者完全康复。对于排除感染性中枢神经系统疾病的局灶性神经功能障碍患者，鉴别诊断时应考虑TTP。
