以神经系统症状为首发表现的血栓性血小板减少性紫癜1例报告并文献复习62例临床分析

目的分析以神经系统症状为首发或主要表现的血栓性血小板减少性紫癜(TTP)的临床特点,提高临床医生尤其是神经科医生对于TTP的认识。方法收集以神经系统症状为首发表现的TTP患者1例,结合国内文献报道62例,对共计63例患者的临床特点进行分析。结果 63例患者中男性25例,女性38例,男∶女=1∶1.52,发病年龄19~89岁,平均发病年龄(45.68±15.79)岁。首发症状主要为意识障碍、发热、头痛、头晕、言语不清及肢体无力。疾病早期曾被诊断为颅内感染、脑卒中、Evans综合征、多发性肌炎。颅脑影像学可表现为正常、脑出血、脑梗死、脑水肿、静脉窦血栓。结论当患者的症状不能完全用脑卒中、颅内感染等神经科疾病解释,尤其伴有血小板减少、贫血等血液系统异常时,要想到TTP,及时完善破碎红细胞、乳酸脱氢酶、肝功能、肾功能、ADAMTS13等化验,及时给予血浆置换往往可以改善患者预后。     

血栓性血小板减少性紫癜的神经系统表现

血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)是一种严重的微血管血栓-出血综合征,其临床主要表现为发热、血小板减少性紫癜、微血管内溶血性贫血、中枢神经系统症状和肾脏损害五联征。多数TTP患者起病急、病情凶险、体征多变,容易被误诊和漏诊,并且     

以神经系统病变为首发表现的血栓性血小板减少性紫癜2例报告

血栓性血小板减少性紫癜 (TTP)是一种少见的严重血液病 ,我院近年来收治 4例 ,其中 2例以神经系统病变为首发表现 ,现报告如下。1　病例　1 1　例 1　男 ,4 4岁。因低热半个月 ,反应迟钝、发作性右侧肢体无力 1周于 1996年 11月 2 1日以“脑血管疾病”收住神经内科。入院前因“感冒”口服感冒通、康泰克等 7天。查体 :T37 8℃ ,P、R、BP均正常。轻度贫血貌 ,两上臂内侧见有小片状瘀斑 ,巩膜不黄 ,心肺、肝脾无异常。言语迟缓、反应迟钝、答非所问。计算力、记忆力明显障碍。脑神经正常 ,右侧肢体肌力 4级 ,右半身痛觉减退、巴氏征阳性 ,…     

以神经系统症状首发的血栓性血小板减少性紫癜一例报道并文献复习

血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TIP)是一种少见的血液系统疾病.虽然该病的发病率相对较低,但多数患者起病急骤,病情凶险,如不能正确诊断并及时有效治疗,病死率极高.部分以神经系统症状首发或主要表现的患者,容易被误诊为脑炎或脑血管病而延误治疗.     

以神经系统症状为主诉的血栓性血小板减少性紫癜:1例报道及文献复习

本文报道了1例以神经功能受损为主诉的血栓性血小板减少性紫癜患者的诊治过程。该患者男性65岁,急性起病,症状表现为失语、肢体麻木及无力,症状波动而多变,并伴有发热。血液学检查发现贫血、血小板计数下降;有轻度肾功能受损;外周血涂片见红细胞碎片;血浆ADAMTS13活性显著降低,ADAMTS13抑制物阳性。该例患者被诊断为特发性血栓性血小板减少性紫癜,经及时诊断及积极进行血浆置换治疗后,病情得以缓解。     

血栓性血小板减少性紫癜神经系统损害的影像学特征

目的探讨具有神经精神表现的急性血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)的神经影像学特点及其对疾病转归的影响。方法回顾性分析2004～2009年广西医科大学第一附属医院神经内科或血液内科住院治疗,具有神经精神症状的急性TTP患者,急性期及随访的头颅CT、MRI等临床资料。结果本组符合条件的患者12例,其中女8例,男4例。神经精神症状以头痛(66.7%)、意识障碍(50%)、精神异常(50%)、失语(25%)等多见;头颅MRI显示11例(91.7%)脑内存在异常病灶,CT显示仅3例(25%)异常。MRI显示病变主要表现为皮质下白质、部分延伸至灰白质交界或皮质的斑片状、边缘欠清的长T1、长T2及T2FLAIR高信号的病灶,部分病灶为混杂信号。随访的MRI显示上述病灶可随症状改善而减少或消失。3例MRI显示为多病灶融合的、大面积多发脑梗死,其中1例伴出血的患者,因病情严重而死亡。结论具有神经精神表现急性TTP的影像学检查,MRI优于CT。其MRI特征性病变为:皮质下白质斑片状、边缘欠清的长T1、长T2及T2FLAIR高信号、部分为混杂信号的病灶,而且上述...     

血栓性血小板减少性紫癜神经系统损害14例临床分析

目的 探讨血栓性血小板减少性紫癜(TTP)神经系统损害的临床特点、诊断及治疗。方法 回顾性分析14例TTP患者的临床资料。结果 本组表现为发热、微血管病性溶血性贫血、血小板减少、神经系统受损、肾功能损害5联征8例，表现为发热、溶血性贫血、血小板减少3联征并神经系统损害6例。神经系统症状均为首发症状之一，以头痛、头昏(10例)最多见，其次为抽搐(9例)，肢体麻木、无力(6例)，感觉异常(3例)，定向障碍、思维混乱、谵妄(6例)、幻觉妄想(1例)、感觉异常(3例)。本组均给予糖皮质激素及抗血小板药物治疗，同时应用血浆置换(PE)治疗9例。结果 痊愈6例(均为PE治疗者)，好转2例，死亡6例。结论，TTP是一种少见的多系统损伤包括脑在内的全身脏器微动脉和毛细血管广泛闭塞的严重疾病。局灶性神经系统损害症状相当常见，早期治疗可降低死亡率，PE是TTP的首选治疗。     

血栓性血小板减少性紫癜临床分析

目的:探讨血栓性血小板减少性紫癜(TTP)的临床特点、诊断要点及治疗方法。方法:分析7例患者的临床特点、血液学特征性改变、影像学特征及实验室检查结果。结果:急性发病,临床表现为贫血、皮肤淤斑、鼻出血、黑便等血液学的异常;头痛、头晕、意识障碍、神经功能缺损或癫痫发作;黄疸、血尿及发热等,实验室检查为溶血性贫血、血小板减少和肾功能异常,神经影像学表现为脑梗死灶或伴出血。在明确诊断后应用激素、血浆置换治疗及对症治疗,7例中2例基本痊愈,2例放弃治疗,3例死亡。结论:对临床急性起病的表现为贫血、出血、头痛、神经功能缺损或癫痫发作的患者,结合血小板减少应高度怀疑TTP,及时进行骨髓像等血液学检查,一旦确诊,及早给予激素、血浆置换治疗及相应对症治疗具有挽救生命的重要意义。     

血栓性血小板减少性紫癜的神经系统特点及治疗

血栓性血小板减少性紫癜（thrombotic thrombocytopenicpurpura,TTP）是一种临床少见病,可以以神经系统表现为首发症状和主要表现,误诊率高,病情凶险,病死率高。我们回顾性分析了我院2001-01~2009-01年确诊的3例TTP患者的临床病历资料,并复习相关文献,以期提高该病的诊治水平。     

表现为持久轻偏瘫的血栓性血小板减少性紫癜

ＴＴＰ是一种少见的，包括脑在内的全身脏器微动脉和毛细血管广泛闭塞的严重疾病。短暂的局灶性神经系统症状相当常见，并常常为首发症状。本文报告一例已妊娠２５周的病例，因突发左侧无力入院，体检为左侧轻瘫。实验室检查包括微血管病性溶血性贫血，抗人球蛋白试验阴性，血小板减少。     

Full neurologic recovery after fulminant thrombotic thrombocytopenic purpura with status epilepticus

Thrombotic thrombocytopenic purpura (TTP) is an ischemic vasculopathy frequently associated with neurological dysfunction including seizures. However, status epilepticus (SE) has rarely been reported in this condition. We report on a 70-year-old woman with fulminant TTP who developed convulsive SE despite high therapeutic serum levels of phenytoin and phenobarbital. Her electroencephalogram (EEG) was characterized by bilateral independent periodic lateralizing epileptiform discharges (BIPLEDs) propagating into clinical and electrographic seizures. She recovered completely after intensive plasmapheresis and treatment with pentobarbital induced coma for 5 days. This case illustrates that aggressive treatment with pentobarbital and plasmapheresis may prevent permanent neurologic deficits when TTP is complicated by SE and that periodic lateralizing epileptiform discharges (PLEDs) in this syndrome can be the manifestation of a reversible ischemic insult.     

Epitope analysis of autoantibodies to ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura

Introduction

Autoantibodies to ADAMTS13 have a pivotal role in the pathogenesis of acquired thrombotic thrombocytopenic purpura (TTP). By decreasing the function of ADAMTS13, autoantibodies impair the cleavage of ultra-large von Willebrand factor (UL-VWF) multimers into smaller sizes, leading to lethal platelet-VWF thrombi in the microcirculation. We therefore aimed to determine the sites of autoantibody recognition on ADAMTS13.

Materials and Methods

In this study, IgG purified from 13 acquired TTP patients were examined to determine their binding sites on ADAMTS13. Immobilized IgG on microtiter plate or proteinG beads was screened by phage library expressing various peptides of ADAMTS13.

Results

In screening, diverse peptide sequences were obtained from almost all of the ADAMTS13 domains, including the spacer domain, which is considered a major binding site. In particular, we detected an identical amino-acid sequence in the C-terminus of the spacer domain from Gly662 to Val687 that was recognized by autoantibodies from 5 TTP patients. The specific autoantibody was expected to be associated with the plasma levels of the ADAMTS13 antigen or activity, and with the quantity of ADAMTS13 autoantibodies or the inhibitory autoantibody titer in TTP patient plasma. These measurements, however, did not seem to be related to the presence or absence of the specific autoantibody.

Conclusions

These findings indicate that the specific autoantibody might be a feature of acquired TTP, although its clinical significance remains to be elucidated.     

Ocular manifestations of thrombotic thrombocytopenic purpura

We report a case of thrombotic thrombocytopenic purpura (TTP) in a 47-year-old woman, who presented fluctuating visual disturbances which had developed over the last six months. An antiphospholipid syndrome was suspected and intravenous heparin treatment was started. One week later, hemolytic anemia and renal insufficiency occurred. Severe deficiency of von Willebrand factor-cleaving protease was found and a diagnostic of TTP was made. The clinical outcome was favorable after treatment with plasmapheresis and fresh frozen plasma. Diagnosis, etiology and treatment of this life-threatening disease are discussed.     

以神经系统症状首发的恶性肿瘤37例临床分析

恶性肿瘤以神经系统症状起病的并非少见,但许多原发肿瘤症状不表现或隐匿.常以神经系统症状就诊从而被误诊.故现将自2001年10月至2006年11月南京军区总医院神经内科收治的以神经系统症状首发且均经CT、MRI或活检等检查后确诊的37例恶性肿瘤患者的临床资料报道如下,以期提高其诊断正确率.     

Thrombotic thrombocytopenic purpura in the brain: possible prognostic impact of von Willebrand factor physiology

We report three patients with CNS involvement in thrombotic thrombocytopenic purpura (TTP). The first two cases presented with fluctuating levels of consciousness and lateralized neurologic deficits. Repeated brain CT scans were normal in case 1, and showed small parietal lucencies in case 2. Both patients recovered to only minor residues after combined treatment of plasma exchange, corticosteroids, immunoglobulins, vincristine (case 1), and cyclophosphamide (case 2), respectively. Case 3 with multiple infarctions in brain CT died of heart failure. Autopsy showed myocardial infarction and multiple hemorrhages in various organs including cerebral cortex. Excess deposition of von Willebrand factor antigen within hyaline thrombi in small and medium-sized arteries of the cerebral cortex is shown for the first time immunohistologically.     

26例脑静脉窦血栓形成临床分析

目的分析脑静脉窦血栓形成(CVST)的病因、临床特点,提高对脑静脉窦血栓形成的诊断和治疗水平。方法回顾性分析2008-01-2016-01我院经头CT、MRV及脑血管造影(DSA)确诊的26例CVST患者,分析其病因、临床症状、腰椎穿刺结果、影像学特点及预后。结果病因有产褥期、妊娠期、长期服避孕药、重度脱水、肺癌、血液病、肥胖、自身免疫性疾病,另有病因不明者。首发症状表现为头痛、视乳头水肿、恶心呕吐、意识障碍、癫痫、偏瘫、发热及复视、视力下降。脑脊液检查示颅压增高、蛋白轻度增高,头CT示弥漫性脑水肿、蛛网膜下腔出血、空三角症、静脉性梗死致出血及未见异常。结论 CVST的临床症状多样化,确诊需影像学检查,积极治疗预后好,以综合治疗为主,包括脱水降颅压、降纤、抗凝及原发病的治疗和血管介入治疗。     

26例脂质沉积性肌病的临床特点

目的 总结脂质沉积性肌病（lipid storage myopathy LSM）的临床特征.方法 回顾性分析本院2009-2013年确诊的26例LSM患者的临床资料.结果 LSM主要临床特点为近端无力和运动不耐受,肌酶谱轻中度升高,肌电图呈肌源性损害或神经源性损害,肌肉MRI检查早期选择性累及大腿后组肌群,尿和血代谢可出现特异性改变,病理检查油红O染色显示空泡为大量脂滴充填,电镜证实肌纤维内脂滴堆积;积极治疗可好转,但病情易反复.结论 LSM临床表现不特异,肌活检是目前诊断LSM的重要方法,LSM患者肌肉MRI、尿和血代谢有特征性改变,基因检查可进一步明确病因.