大动脉炎引起可逆性后部白质脑病综合征一例

<正>可逆性后部白质脑病综合征(posterior reversible encephalopathy syndrome,PRES)是一组急性起病,以头痛、精神改变、癫痫发作、视力下降为主要临床表现,神经影像学表现为可逆性大脑后部(以顶枕叶为主)白质损害的临床综合征。临床上PRES并不少见,病因多样。而大动脉炎(Takayasu′s arteritis)作为一种血管炎性病变,可导致PRES发生,临床上非常少见。1病例报告患者27岁女性,因突发头痛、视物不清12h于2015-8-13入院。患者12h前睡眠中突发头痛,为头顶部持续性疼痛,性质不能描述,伴视物不清、头晕、恶心呕     

可逆性后部白质脑病综合征

可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)最早是由Hinchey等[1]在1996年提出,近年逐渐被临床医生认识.尽管其命名和发病机制尚存在许多争论[1-4],但其临床及影像学有着特征性.本人复习文献,结合临床体会,对RPLS的病因、发病机制、临床表现、影像学特征、诊断、治疗及转归进行综述.     

可逆性后部脑病综合征

可逆性后部脑病综合征自1996年首先提出至今已有12年,早先国外报道较多,国内随着对该病的认识及影像学技术特别是MRI技术的进步,临床发现该病逐渐增多,近年来报道也越来越多。     

肾动脉狭窄致可逆性后部脑病综合征1例

1 病例报告 患者女,18岁,因头痛伴恶心呕吐5d入院.患者5d前无诱因出现头痛,部位为全头部,钝痛,呈持续性发作,疼痛较剧烈,伴恶心呕吐.发病时无发热,肢体抽搐及意识障碍.在当地医院应用甘露醇后症状可暂时好转.既往身体健康,无高血压、心脏病、肾脏病、头痛病史.     

可逆性后部脑病综合征9例临床分析

目的：探讨可逆性后部白质脑病综合征的临床特点,以提高该病临床诊疗水平。方法回顾性分析我院2009-11-2013-06共9例可逆性后部白质脑病综合征病人的临床资料。结果9例可逆性后部白质脑病综合征病人中7例为妊娠高血压综合征、先兆子痫;2例分别为慢肾、尿毒症、肾性高血压及原发性高血压。经及时有效治疗后完全恢复。结论可逆性后部白质脑病综合征多发生在妊娠高血压综合征、先兆子痫,急性起病或亚急性起病;以头痛、头晕、视物模糊、癫痫样发作、意识障碍为主要表现。其影像学特征性改变为异常的相对对称性的枕顶叶病灶。及时诊治病情可逆。     

可逆性后部白质脑病综合征临床探讨

目的探讨可逆性后部由质性脑病综合征（RPLS）的病因、临床表现、影像学特征及治疗。方法总结分析8例可逆性后部白质脑病综合征患者的发病原因、临床症状、影像学检查特点、治疗及预后。结果8例患者经针对病因治疗（如降压、止痉、停止使用免疫抑制药物、化疗药、停止放疗、控制肺部感染）及酌情使用甘露醇、呋噻米等脱水降颅压药物,症状与体征恢复至发病前6例,基本消失2例。结论可逆性后部白质脑病综合征是一种有特征临床和影像学检查表现的临床综合征,及时就诊、尽早治疗,一般预后良好。     

以可逆性皮层盲为主要表现的可逆性后部白质脑病2例临床分析

目的 探讨以可逆性皮层盲为主要表现的可逆性后部白质脑病的临床特点、影像学表现、诊断和治疗。方法 回顾性分析主要表现为可逆性皮层盲的2例患者的临床资料。结果 2例均合并妊娠子痫,均伴有高血压、头痛、癫痫,1例合并蛛网膜下腔出血,1例合并HELLP综合症,头颅核磁共振均提示血管源性脑水肿,表现为双侧枕叶皮质、皮质下白质T₁低信号、T₂高信号、DWI低信号或等信号,MRA、MRV正常,经积极脱水降颅内压、控制血压等对症治疗后效果良好。结论 可逆性皮层盲是可逆性后部白质脑病的特征性表现之一,早期明确诊断、及时充分控制血压及脱水降颅内压、止抽等积极对症治疗对患者预后有重要作用。     

妊娠相关可逆性后部脑病综合征36例临床分析

目的探讨妊娠相关可逆性后部脑病综合征(posterior reversible encephalopathy syndrome,PRES)的临床及影像学特点。方法检索2010年1月-2016年6月在湘雅医院妇产科和神经内科住院的所有行头部影像学检查的子痫前期/子痫/PRES患者,进一步从中筛选出妊娠相关的PRES患者,并总结其临床特点、影像学资料及治疗经过。结果本组共检索到90例子痫前期/子痫/PRES患者,最终确诊的妊娠相关PRES患者36例。28例子痫患者中有26例(92%)被确诊为PRES。最常见的临床表现是头痛(81%),其次依次为癫痫发作(73%)、意识状态改变(57%)、恶心/呕吐(47%)、视物模糊(33%)及局灶性神经功能缺损等。PRES的典型影像学表现为大脑后部为主的皮质和皮质下可逆性血管源性水肿,不典型的累及部位包括额叶(72%)、颞叶(67%)、基底节(50%)、小脑(47%)、脑干(14%)和丘脑(8%)。不典型的影像学特点包括弥散受限(33%)、出血(19%)和对比强化(16%)。并发症包括血小板减少(25%)、肺部感染(25%)、贫血(19%)、发热(17%)、急性肾衰竭(8%)、HELLP综合征(6%)及DIC(3%)。大部分患者经积极诊治后可明显好转,但有1例并发DIC的患者最终死亡。结论大部分子痫患者可能进一步诊断为PRES;妊娠相关PRES临床及影像学表现多样,提高对该疾病的认识具有重要临床意义。     

可逆性后部白质脑病综合征

可逆性后部白质脑病综合征(RPLS)是一组由多种原因引起的以神经系统异常为主要表现的综合征,临床表现以迅速进展的颅高压症状、癫发作、视觉障碍、意识障碍、精神异常为特征,神经影像学上显示以双侧大脑后部白质为主的水肿区,经及时有效治疗后临床表现和神经影像学改变可以完全恢复,一般不遗留有神经系统后遗症。核磁共振新技术的发展,突出显示了血管源性水肿的特点,有助于RPLS的正确诊断。     

酷似脑干肿瘤的可逆性后部白质脑病综合征一例

可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)是由多种病因引起的可逆性大脑后部灰白质受累为主要特征的一种临床综合征,多累及双侧顶枕叶,也可累及其他部位,以脑干受累为主的RPLS称作脑干型RPLS,相对罕见.现报道1例酷似肿瘤的脑干型RPLS病例.1 病例报告 患者男,64岁,因头晕、乏力2.5个月,复视半个月,加重10 d于2013-05-03收入院.入院前2.5个月患者出现乏力,伴头晕、头胀,未给予特殊处理.后出现一过性复视.10 d前患者无明显诱因感觉头晕加重,步态不稳.     

阿帕替尼导致可逆性后部脑病综合征1例

正1病例介绍患者女性,56岁,主因"头痛伴间断性抽搐6 d"以症状性癫痫于2016年8月26日收入血管神经病学病区。患者于6 d前无明显诱因出现头痛、视物模糊,继而出现意识丧失、四肢抽搐、双眼上翻、口角向右歪斜,持续5 min后症状缓解,予镇静对症治疗,期间抽搐发作6次。后患者无抽搐发作,但头痛持续不缓解,遂收入院     

Posterior Reversible Encephalopathy Syndrome Associated with Hemorrhage

Background  Posterior Reversible Encephalopathy Syndrome (PRES) is a clinico-radiological entity characterized by headache, encephalopathy, visual disturbances, and seizures in association with reversible vasogenic edema on neuroimaging. Intracerebral hemorrhage associated with PRES (PRES-ICH) is generally considered an atypical finding. Methods  Retrospective case series. Results  Seven patients were identified with PRES-ICH, four males and three females. The presenting clinical symptoms included headache (2), encephalopathy (5), vision changes (2), seizures (2), and hemiparesis (1). The co-morbid conditions included acute renal dysfunction (3), solid organ transplantation (3), bone marrow transplant (1), use of calcineurin inhibitor agents (4), and pre-eclampsia (1). Neuroimaging revealed intraparenchymal hemorrhage in six patients and subarachnoid hemorrhage in one patient. Six of the seven patients with PRES-related ICH had underlying bleeding diathesis, including one patient who was anti-coagulated with a heparin drip. The mean platelet count was 82.1 × 10³/μl (range, 4–232 × 10³/μl), and the mean INR value was 2.18 (range, 0.9–6.7). Two patients died during the acute hospitalization. Among the five survivors, only two patients had good functional outcome (pre-defined as mRS ≤ 1). Conclusion  In our series, the majority of patients with PRES-ICH (85%) had an underlying bleeding diathesis or coagulopathy. Although PRES is typically considered to have a favorable prognosis, the clinical outcome of PRES with associated ICH can be more variable.     

Refractory Intracranial Hypertension in Posterior Reversible Encephalopathy Syndrome

Introduction

Posterior reversible encephalopathy syndrome (PRES) is a largely reversible disease with long-term favorable outcome. A minority of patients, however, may develop progressive cerebral edema and ischemia resulting in severe disability or death. We report a case of severe intracranial hypertension associated with PRES that was successfully treated according to intracranial pressure (ICP)- and cerebral perfusion pressure (CPP)-driven therapy.

Methods

Case report.

Results

A 42-year-old woman underwent bilateral lung transplantation for severe bronchiectasis. Her immunosuppressive regimen consisted of azathioprine, prednisone, and tacrolimus. She acutely developed an aggressive form of PRES that rapidly resulted in severe refractory intracranial hypertension despite discontinuation of potentially causative medications and adequate supportive therapy. Accordingly, second-tier therapies, including barbiturate infusion, were instituted and immunosuppression was switched to anti-thymocyte globulin followed by mycophenolate mofetil. Within 10 h of barbiturate administration, ICP dropped to 20 mmHg. Thiopental was administered for two days and then rapidly tapered because of severe urosepsis. Six months after discharge from the intensive care unit the patient returned to near-normal life, her only complaint being short-term amnesia.

Conclusions

The decision to undertake ICP monitoring in medical conditions in which no clear recommendations exist greatly relies on physicians’ judgment. This case suggests that ICP monitoring may be considered in the setting of acute PRES among selected patients, when severe intracranial hypertension is suspected, provided that a multidisciplinary team of neurocritical care specialists is readily available.     

Posterior Reversible Encephalopathy Syndrome Following a Scorpion Sting

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic entity not yet understood, that is present with transient neurologic symptoms and particular radiological findings. The most common imaging pattern in PRES is the presence of edema in the white matter of the posterior portions of both cerebral hemispheres. The cause of PRES is unclear. We report a case of 13‐year‐old male who was stung by a scorpion and developed a severe headche, visual disturbance, and seizures and had the diagnosis of PRES with a good outcome. Numerous factors can trigger this syndrome, most commonly: acute elevation of blood pressure, abnormal renal function, and immunosuppressive therapy. There are many cases described showing the relationship between PRES and eclampsia, transplantation, neoplasia and chemotherapy treatment, systemic infections, renal disease acute, or chronic. However, this is the first case of PRES following a scorpion sting.     

Isolated Leptomeningeal Enhancement in Tacrolimus-Associated Posterior Reversible Encephalopathy Syndrome

We report on tacrolimus-associated posterior reversible encephalopathy syndrome with the previously unreported finding of leptomeningeal enhancement occurring separate from the site of parenchymal magnetic resonance signal abnormality. Recognition of this atypical finding as a noninfectious cause of leptomeningeal enhancement may assist those caring for patients affected by posterior reversible encephalopathy syndrome.