可逆性后部白质脑病综合征临床及影像学特点分析

目的 探讨可逆性后部白质脑病综合征(RPLS)的临床和影像学特点.方法 回顾性分析9例RPLS患者的临床及影像学资料.结果 9例患者临床表现以头痛、意识障碍、癫发作、视觉异常为主;头颅MRI检查有大脑半球后部白质为主的T1WI低信号,T2WI、Fair像呈高信号的病灶,且大多双侧对称.结论 头痛、意识及精神障碍、癫发作、视觉异常、是可逆性后部白质脑病综合征主要临床表现,影像学特征主要为大脑后部对称性、可逆性脑白质损害.     

可逆性后部白质脑病综合征临床影像学特征

目的探讨可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)的临床和影像学特点。方法回顾性分析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、影像学特征、治疗及预后。结果8例患者中,女5例,男3例;发病原因多样,系统性红斑狼疮2例,妊娠子痫2例,高血压病1例,真菌性脑炎1例,多发性骨髓瘤1例,肾移植1例;其中使用免疫抑制剂3例,化疗药物1例,使用抗真菌药物1例。以头痛、癫痫发作、视觉障碍、意识和精神障碍为主要临床症状。多数患者头颅MRI表现为双侧大脑后部白质对称性T1WI呈低或等信号,T2WI和FLAIR像呈高信号;经采取降压、脱水及对症等治疗2~3周后患者神经系统症状消失,MRI复查病灶完全或大部分消失。结论RPLS是以可逆性后部白质损害为主要神经影像学表现的临床综合征,及时、准确的诊断和治疗可使其临床症状和影像学改变完全可逆。     

可逆性后部白质脑病综合征临床及影像学特点15例分析

目的探讨可逆性后部白质脑病综合征的临床和影像学特征。方法分析15例可逆性后部白质脑病综合征患者的临床和影像学资料。结果 15例可逆性后部白质脑病综合征患者继发于高血压脑病、系统性红斑狼疮、尿毒症、子痫、甲状旁腺腺瘤伴发高钙血症、非霍奇金淋巴瘤、肾移植术后、急性淋巴细胞白血病和神经白塞病。其中血压升高者10例,临床主要表现为头痛、癫痫发作、视物模糊和意识障碍。头颅CT或MRI检查主要表现为对称的枕叶白质脑水肿,部分伴有顶额颞叶等病变。经治疗后患者症状消失,头颅CT或MRI复查病灶完全或大部分消失。结论可逆性后部白质脑病综合征可由多种病因产生,影像学主要表现为大脑后部白质血管源性脑水肿改变,及时治疗后症状和影像学特征迅速改善。     

可逆性后部白质脑病综合征合并HELLP综合征的临床分析

目的分析可逆性后部白质脑病综合征(PRIS)合并HELLP综合征患者的临床特征,提高对该病的认识。方法回顾性分析我院诊治1例及国内文献报道4例.PRLS合并HELLP综合征的临床表现、实验室及影像学检查、治疗和转归。结果5例患者中出现严重高血压4例,头痛5例,癫痫发作5例,视觉异常5例,意识障碍5例,精神障碍2例,局灶性神经系统定位体征3例,肢体水肿1例。实验室检查均提示有溶血、肝酶升高、血小板减少。5例患者头部cT或MRI检查均显示双侧顶枕叶白质为主的对称性异常,部分累及额叶、颞叶,病灶在cT为低密度灶,MRI为长T_1长T_2信号灶。结论 RPLS同时合并HELLP综合征是妊高征患者罕见的并发症,充分认识其临床和影像学特点,有助于及时诊断和治疗。     

流行性出血热合并可逆性后部白质脑病综合征1例报告

<正>可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)是一组近几年才被临床医生逐渐认识的神经影像学综合征,它以头痛、癫痫发作、视觉障碍、意识改变以及精神行为异常为主要临床表现,神经影像学主要表现为可逆性大脑后部白质损害,随着临床病例的增多以及研究的进展,目前发现本病也可累及脑干、小脑和基底核。本文报道1例流行性出血热并发RPLS,并根据临床     

可逆性后部白质脑病

可逆性后部白质脑病(reversibleposteriorleukoencephalopathysyndrome,RPLS),1996年被Hinchey等[1]首次报道,又称后部可逆性脑病综合征(PRES)、高灌注性脑病、顶枕叶脑病、可逆性后部脑水肿综合征,是一种临床-影像综合征,典型的临床症候群包括:头痛、意识障碍或精神行为异常、癫痫发作和视觉障碍.     

子癎前期、子癎患者并发可逆性后部白质脑病综合征的MRI表现及临床分析

目的探讨子癎前期、子癎并发可逆性后部白质脑病综合征(RPLS)的MRI表现及临床特点。方法回顾性分析17例子癎前期、子癎并发RPLS患者的临床资料。结果 17例RPLS均有高血压、头痛,14例恶心、呕吐;13例视力障碍;16例子癎发作;MRI检查大脑后部为主的白质水肿,特别是枕叶,T2WI、FLAIR呈高信号,DWI呈等信号或稍低信号,ADC图呈高信号。结论子癎前期、子癎易并发可逆性后部白质脑病综合征,视力障碍是RPLS的特征表现,MRI检查可明确诊断RPLS。     

可逆性后部白质脑病2例临床分析

目的 探讨可逆性后部白质性脑病综合征（reversible posterior leukoencephalopathy syndrome，RPLS）的病因、临床表现、影像学特征及治疗。方法 分析2例可逆性后部白质脑病综合征患者的临床资料，并复习文献。结果 2例患者均有头痛、视物不清、头部MRI检查示T2高信号，其中1例伴有癫发作，经治疗患者的临床表现及影像学均很快好转。结论 可逆性后部白质脑病综合征的临床表现无特异性，经对症处理后临床及影像学表现均很快好转。     

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。     

围产期可逆性后部白质脑病一例

目的探讨围产期可逆性后部白质脑病(RPLS)的病因,临床表现,影像学表现。方法介绍本院收治的1例围产期可逆性后部白质脑病的临床表现,影像学表现及治疗。结果本例临床症状主要表现为头痛、迅速进展的高颅压症状、癫痫发作、视觉障碍和精神异常,影像学表现主要为以额顶叶为主的可逆性损害,给予控制癫痫,调整血压,减轻脑水肿,抗感染等治疗后患者临床症状及影像学表现均恢复。结论围产期RPLS临床表现多种多样,主要表现为头痛、迅速进展的高颅压症状、癫痫发作、视觉障碍和精神异常,影像学表现主要为以额顶叶为主的可逆性损害,需要与静脉窦血栓,中枢神经系统感染等鉴别,治疗效果及预后良好。     

Interictal cortical reorganization in episodic migraine without aura: an event-related fMRI study during parametric trigeminal nociceptive stimulation

Reversible posterior leukoencephalopathy syndrome (RPLS) is theoretically associated with hypertensive encephalopathy because the most patients demonstrate abrupt increasing of blood pressure (BP). A 59-year-old woman, who had undergone cholecystectomy 4 days before, complained of a headache and rapidly progressing visual disturbance. Her BP was postoperatively controlled at around 150/80, but her BP was 89/46 when she noticed her symptoms. Magnetic resonance imaging showed vasogenic edema in bilateral occipital and right parietal lobes, and intracranial magnetic resonance angiography revealed bilateral diffuse peripheral vasoconstriction. After discontinuing ropivacaine administration via epidural catheter, her BP rose to 114/62 and her symptoms completely disappeared within a few days. Except for hypotension, the clinical course and the radiological evidences in our case were consistent with RPLE. This case supports another hypothesis of RPLS mechanism that arterial endothelial injury by toxic drug effect results in transudation of fluid from blood vessels causing vasogenic brain edema.     

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。     

以可逆性皮层盲为主要表现的可逆性后部白质脑病综合征三例临床和影像学分析

目的探讨主要表现为皮层盲的可逆性后部白质脑病综合征的临床与影像学表现、早期诊断和治疗。方法回顾性分析主要表现为可逆性皮层盲的慢性肾功能衰竭患者1例、先兆子痫和子痫患者各1例的临床、腰椎穿刺和影像学资料。结果3例患者均出现急性双侧完全性皮层盲,伴有高血压、头痛,1例伴有癫痫发作。腰椎穿刺示轻度颅内压增高。头颅MRI示双侧枕叶皮层或皮层下对称性异常信号,T2WI、FLAIR为高信号,DWI为低或等信号,ADC为高信号,提示血管源性水肿。3例患者均诊断为可逆性后部白质脑病综合征,经控制血压、脱水降颅压等治疗,皮层盲在2天内均完全好转,2周后复查头MRI病灶完全消失。结论可逆性皮层盲是可逆性后部白质脑病综合征最为特征性的症状之一,是由于双侧枕叶皮层或皮层下血管源性水肿所致。     

可逆性后部白质脑病综合征57例临床分析并文献复习

目的 探讨可逆性后部白质脑病综合征(RPLS)的临床和影像学特征.方法 回顾性分析57例(我院3例,文献54例)RPLS患者的临床资料.结果 本组成人多见,女性比例偏高.继发于先兆子痫/子痫19例(33.3%),高血压病18例(31.6%),肾功不全10例(17.5%),其他原因为红斑狼疮、应用化疗药和细胞毒性药、血管炎、肾病综合征、急性肾小球肾炎.头痛、癫痫发作、意识障碍、视力障碍是RPLS常见四联征,54例伴急性血压升高.影像学表现为大脑后部对称性白质水肿,CT低密度灶,MRIT1低信号,T2和Flair像高信号.50例累及双侧顶枕叶,3例累及单侧枕叶,其他部位受累频率依次为额叶、颞叶、小脑、基底节区、脑干、丘脑、胼胝体.经过治疗,55例患者临床症状、影像学异常迅速恢复.结论 头痛、癫痫、意识障碍、视力障碍是RPLS主要临床表现,血压急性升高是RPLS的重要体征,影像学特征是大脑后部对称性白质水肿,大多数患者经治疗很快恢复.

Abstract：

Objective To investigate the clinical and imaging characteristics of posterior reversible encephalopathy syndrome (RPLS). Methods The clinical data of 57 patients with RPLS were analyzed retrospectively ( 3 patients from our hospital and 54 from reference). Results Among the 57 cases with RPLS,most patients' symptoms occurrenced at adults and females cases were more than males. 19 cases(33.3% ) were secondary to preeclampsia/eclampsia, 18 cases (31.6%) were secondary to hypertension, 10 cases( 17.5% ) were secondary to renal inadequacy,other reasons were lupus erythematosus, application of chemotherapeutics, cytotoxic drug, vasculitis, nephrotic syndrome and acute glomerulonephritis.The common tetrad are headache, epileptic attack, conscious disturbance and visual disorder. 54 cases were accompanied with acute hypertension. Radiologic findings showed edema predominantly in the posterior cerebral white matter bilaterally,low density in CT, low signal in MRIT1, High signal in T2 and Flair. The bilateral apical and occipital lobe was involved in 50 cases,lateral occipital lobe was involved in 3 cases, other parts are frontal lobe, temporal lobe, cerebellum, basal ganglia,brain stem, thalamus, callosum. The clinical manifestations and imaging lesions of 55 cases were both improved soon after treatment. Conclusions The main clinical manifestations of RPLS are headache, epileptic attack, conscious disturbance and visual disorder. The important physical sign of RPLS is acute hypertension. Radiologic characteristic is edema predominantly in the posterior cerebral white matter bilaterally, and most patients snap back after treatment.     

Neurogenic Stunned Myocardium Associated with Reversible Posterior Leukoencephalopathy Syndrome

Introduction  Neurogenic stunned myocardium is characterized by transient left ventricular systolic dysfunction in the absence of significant obstructive coronary artery disease. Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by transient vasogenic subcortical edema without infarction. Both syndromes are hypothesized to result from sympathetic dysregulation. We report a case of neurogenic-stunned myocardium and RPLS occurring simultaneously in a patient. Methods  Single case report. Results  We present a 55-year-old woman with a history of hypertension and chronic back pain status post spinal cord stimulator who presented with severe headache, seizure, and confusion associated with acute hypertension. Magnetic resonance imaging (MRI) of the brain revealed bilateral patchy T₂ signal hyperintensity, consistent with RPLS. Transthoracic echocardiogram (TTE) showed regional-wall motion abnormalities in the apical regions, consistent with neurogenic-stunned myocardium. The patient’s TTE and MRI abnormalities resolved on follow-up studies. Conclusion  Neurogenic-stunned myocardium and RPLS are two reversible clinical syndromes that are hypothesized to be a result of sympathetic dysregulation. Our case suggests that these two syndromes may occur together in the same patient.     

可逆性后部白质脑病综合征的临床及影像学特点

目的探讨可逆性后部白质脑病综合征(RPLS)的临床及影像学特点。方法回顾性分析16例RPLS患者的临床资料。结果患者均为急性起病,其中继发于高血压脑病7例,妊娠子痫9例。患者均出现血压明显升高,其中出现头痛9例,意识障碍9例,癫痫发作13例,视力模糊6例。16例患者MRI均表现为双侧枕叶和/或额、颞、顶叶对称或不对称的T1低信号,T2高信号;其中累及顶叶13例(81.3%),枕叶12例(75.0%),额叶9例(56.3%),颞叶5例(31.3%),小脑4例(25.0%)。弥散加权成像(DWI)显示等/低信号14例,异常高信号2例。所有患者经对症治疗均痊愈。结论 RPLS以血压升高、头痛、视觉异常、意识障碍、癫痫发作为主要临床表现。MRI表现双侧枕叶和/或额、颞、顶叶对称或不对称的T1低信号,T2高信号,经治疗预后良好。     

Reversible posterior leukoencephalopathy syndrome in two patients with microscopic polyarteritis nodosa]

We report here two cases (72-year-old woman, 77-year-old-woman) with perinuclear anti-neutrophil antibody (P-ANCA) positive microscopic polyarteritis nodosa (mPN). Both patients presented with generalized convulsion, consciousness disturbance and severe hypertension several days after the administration of high dose methylpredonisolone followed by oral predonisolone. CT brain scan showed hypodensity area on bilateral posterior lobes. MRI T2 weighted image and FLAIR image showed increased signal intensity on the occipital gray and white matter. Although diffusion weighted MRI disclosed slightly high signal intensity, apparent diffusion coefficient (ADC) value at the occipital gray and white matter remained normal or even elevated. These findings, which were consistent with vasogenic edema, lead us to diagnose them as suffering from reversible posterior leukoencephalopathy syndrome (RPLS). After the second administration of high dose methylpredonisolone, their symptoms and signs, together with the MRI findings, gradually improved. To our knowledge, this is the first report concerning RPLS in a patient with mPN.     

Presentation of reversible posterior leukoencephalopathy syndrome in patients on calcineurin inhibitors

Background

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinico-radiologic diagnosis associated with numerous medical conditions including hypertension, immunosuppressant medications, and eclampsia. It is characterized by headache, altered mental status, seizures, visual disturbance, and neuroimaging consistent with posterior-predominant vasogenic edema.The objective of this study was to characterize the clinical spectrum and outcomes in a large series of RPLS patients, and to compare the presentation of patients taking calcineurin inhibitors (CNIs) to that of other RPLS patients.

Methods

We reviewed records of patients seen by the neurology and transplant services over an 18-year period. Comorbid conditions, medications, blood pressure, laboratory testing, clinical outcomes, and radiographic findings were collected.

Results

84 episodes of RPLS were identified in 79 patients. Etiologies included CNIs (43%), hypertension (29%), renal disease (12%), preeclampsia/eclampsia (7%), and chemotherapy (5%). Patients on CNIs had lower blood pressures (p = 0.002) and a lower prevalence of headache (p = 0.02) compared to RPLS patients with other etiologies. Clinical recovery occurred in 65% of episodes, and radiographic resolution occurred in 67%.

Conclusions

Patients with CNI-induced RPLS have lower blood pressure than other RPLS patients, but otherwise present similarly. RPLS typically occurs within days to weeks of CNI initiation in patients without elevated medication levels. Clinical and radiographic recovery occurred in the majority of patients in this series, but one-third suffered residual neurologic deficits or death. These findings highlight the importance of prompt recognition and treatment of RPLS triggers to prevent permanent sequelae.     

31-year-old gravid woman of reversible posterior leukoencephalopathy syndrome associated with HELLP syndrome after labor]

We report on a 31-year-old gravid woman with reversible posterior leukoencephalopathy syndrome (RPLS) associated with HELLP syndrome. The patient was hospitalized and underwent an emergency cesarean section at the 34th week of pregnancy due to severe toxemia. After labor her blood pressure acutely increased to 180/100 mmHg and a generalized convulsion occurred. Laboratory data revealed hemolysis, elevated liver enzymes, and low platelets, consistent with HELLP syndrome. FLAIR and Apparent Diffusion Coefficient images on MRI showed hyperintense lesions bilaterally in the posterior white matter, however Diffusion-Weighted images revealed slightly hyperintense changes in the same locations. With the use of diltiazem for blood pressure control, and anticonvulsants, convulsions disappeared and consciousness level improved. Moreover the MRI abnormalities also improved after therapy. This case was diagnosed as RPLS associated with HELLP syndrome and is the first case of an investigation utilizing Diffusion-Weighted and Apparent Diffusion Coefficient images to establish this diagnosis. Based on these results, possible mechanisms of RPLS may be vasogenic edema mediated by a cerebrovascular endothelial disturbance of cerebral vessels and a rapid blood-pressure increase due to HELLP syndrome.     

Reversible posterior leukoencephalopathy syndrome: experience in 3 cases]

We report 3 cases with reversible posterior leukoencephalopathy syndrome (RPLS) accompanied by eclampsia or hypertensive encephalopathy. RPLS may develop in patients who have eclampsia or hypertensive encephalopathy or who are immunosuppressed. The findings on neuroimaging are characteristic of subcortical edema without infarction. A 27-year-old primigravida developed eclampsia at 37 weeks of gestation. MRI was performed 4 hours after the onset. The FLAIR sequence delineated extensive hyperintense lesions in the temporal and occipital lobe bilaterally. MR angiography(MRA) performed 6 days after the onset of symptoms clearly demonstrated intracranial vasospasm. Follow up MRI and MRA were performed 3 weeks after the onset. The MRI showed slight residual hyperintensity in the occipital lobe. The MRA showed the disappearance of the vasospasm. A 39-year-old woman on the 8th postpartum day presented with thunderclap headache, which led to a search for SAH. She visited our hospital, whose high arterial blood pressure (220/110 mmHg) was observed. Both CT and MRA were normal. MRI revealed abnormalities in the parieto-occipital regions bilaterally. Treatment of hypertension led to resolution of the posterior leukoencephalopathy. A 38-year-old woman on the 11th postpartum day suddenly developed vertigo, visual disturbance and generalized convulsion. MRI was performed 7 days after the onset. The FLAIR sequence delineated extensive hyperintense lesions in the occipital lobe bilaterally. MRA clearly demonstrated diffuse intracranial vasospasm. MRA performed 3 weeks after the onset showed the disappearance of the vasospasm. In conclusion, our experience suggests that the MRI and MRA noninvasively provide valuable findings which are complementary in the diagnosis and follow-up examination of a brain edema and vasospasm in RPLS.