de Jong F J, Te Boekhorst P A W, Dippel D W J, Jacobs B C
Department of Neurology, Erasmus Medical Center, Rotterdam, The Netherlands.
BMJ Case Rep. 2010 Nov 23;2010:bcr0620103113. doi: 10.1136/bcr.06.2010.3113.
Thrombotic thrombocytopenic purpura (TTP) is characterised by a thrombotic, haemolytic microangiopathy leading to microvascular occlusion, haemolysis and ischaemic dysfunction of various organs including the brain. TTP may present with a variety of neurological symptoms, including headache, focal deficits, seizures and coma. The authors describe a 55-year-old man presenting with abdominal pain and rapidly progressive deterioration into coma without focal neurological deficits or seizures. A concomitant, transient, rapid increase in blood pressure raised the suspicion of a hypertensive crisis. Yet, our patient did not improve after vigorous treatment with antihypertensives. Brain imaging excluded a hypertensive leucoencephalopathy. Despite the absence of a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13) deficiency, the diagnosis idiopathic TTP was made after excluding secondary causes of TTP. Upon treatment with plasma exchange, corticosteroids and vincristin our patient gradually improved. On discharge to a rehabilitation centre he was awake and alert, had minor cognitive deficits and a mild proximal tetraparesis consistent with a critical illness poly(neuro)myopathy.
血栓性血小板减少性紫癜（TTP）的特征是血栓性、溶血性微血管病，可导致微血管闭塞、溶血以及包括脑在内的各种器官的缺血性功能障碍。TTP可能表现出多种神经系统症状，包括头痛、局灶性神经功能缺损、癫痫发作和昏迷。作者描述了一名55岁男性，表现为腹痛，迅速进展为昏迷，无局灶性神经功能缺损或癫痫发作。同时出现的短暂性血压快速升高引发了对高血压危象的怀疑。然而，我们的患者在积极使用抗高血压药物治疗后并未改善。脑部影像学检查排除了高血压性白质脑病。尽管缺乏具有血小板反应蛋白1型基序的去整合素和金属蛋白酶13（ADAMTS13）缺乏，但在排除TTP的继发原因后，仍诊断为特发性TTP。经血浆置换、皮质类固醇和长春新碱治疗后，我们的患者逐渐好转。出院到康复中心时，他清醒且警觉，有轻微认知缺陷和轻度近端四肢轻瘫，符合危重病性多（神经）肌病。
