可逆性后部白质脑病综合征的临床和影像学特征

目的探讨可逆性后部白质脑病综合征(RPLS)的临床特点及影像学特征。方法回顾性总结分析本院2000年9月~2010年6月收治的12例可逆性后部白质脑病综合征患者的临床和影像学资料。结果本组12例患者中原发病为子间痫3例(25%),高血压脑病4例(33.3%),尿毒症2例(16.7%),系统性红斑狼疮2例(16.7%),肺癌术后化疗1例(8.3%),临床表现多样,主要为头痛、呕吐、意识障碍、抽搐、眼震、视力异常等。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。经脱水、降低颅内压、加强补液等对症支持治疗2~3周后神经系统症状消失,复查MRI病灶完全或大部分消失。结论可逆性后部白质脑病综合征的临床特点和影像学具有一定特征性,提高对本病的认识,早期明确诊断十分重要,经及时治疗后一般预后良好。     

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。     

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。     

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的临床与影像学特征

目的探讨可逆性后部白质脑病(reversible posterior leukoencephalopathy syndrome,RPLS)的临床及影像学特点。方法回顾性分析12例可逆性后部白质脑病患者的临床资料,总结其临床及影像学资料。结果本组女8例,男4例。子痫/先兆子痫6例,急性淋巴细胞白血病1例,肾病综合征2例,系统性红斑狼疮1例,再生障碍性贫血1例,原发性高血压病1例。12例患者均急性或亚急性起病,多以头晕头痛、视物模糊或意识障碍为首发症状。2例行腰穿检查,脑脊液细胞数正常、蛋白正常或轻度升高。11例患者行头颅MRI检查,病灶多以双侧枕、顶叶白质为主,部分可累及额颞叶;11例患者病灶均为等或长T1WI信号,长T2WI信号;5例行Flair检查均为高信号;4例DWI为低信号、ADC高信号,增强未见强化。经降压/对症支持治疗后,6例患者头颅MRI随访病灶明显改善或消失。10例患者症状改善或明显缓解。1例失访,1例随访死亡。结论 RPLS的原发病复杂多样,早期治疗临床症状及影像学表现多数可逆,预后良好。     

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。     

可逆性后部白质脑病二例临床分析

目的探讨可逆性后部白质脑病综合征(RPLS)的病因、临床表现、影像学特征、诊断及治疗。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。结果2例患者均急性起病,以头痛、频繁癫发作为首发症状;均发病于产褥期前后,均有血压升高、头晕、视物模糊等症状。1例烦躁、计算、近记忆力下降;另1例恶心、呕吐;头部CT检查2例均显示对称分布于枕、顶、额、颞叶沟回状低密度灶,其中1例广泛脑白质水肿;MRI检查2例均显示对称分布于枕、顶、额、颞叶回状或沟回弥漫片状长T2、长T1信号、Flair像示皮层下弓状纤维形高信号病灶,以脑白质为主、皮质亦见受累。经治疗2例患者的临床症状约1周消失,复查影像学恢复正常。结论头痛、视觉障碍和癫发作是RPLS主要临床表现,影像学特征主要为大脑后部白质对称性CT呈低密度灶、MRI呈长T1、长T2信号。经过正确的治疗,患者的症状、体征及神经影像学改变均可很快好转并可以完全恢复。     

表现为可逆性后部白质脑病综合征的急性间歇性卟啉病(附1例报告)

目的探讨表现为可逆性白质脑病综合征(RPLS)的急性间歇性卟啉病(AIP)的临床特点。方法回顾性分析1例表现为RPLS的AIP患者的临床资料。结果本例患者为青年女性,临床表现为腹痛、癫痫发作、精神症状、植物神经受累表现,检查发现低钠、低氯血症,尿卟啉定性阳性;头颅MRI示两侧额叶后部、顶叶、颞叶皮质和两侧小脑半球弥漫对称性片状异常信号,T1WI为等或低信号,T2WI及Flair序列呈高信号。经高糖、三磷酸腺苷以及纠正电解质紊乱等治疗后症状改善。复查头颅MRI示病变基本消失。结论AIP是RPLS的罕见原因之一。表现为RPLS的AIP可有腹痛和神经精神症状等,尿检和影像学检查对诊断有帮助。     

可逆性后部白质脑病综合征的临床和影像学特征(附2例报告)

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2例RPLS患者的临床资料。结果 2例均为急性起病,例1为产后2 d出现持续高血压,以癫疒间发作、意识障碍为主要症状;例2遭雷击后出现以声音嘶哑、共济失调为主要症状。颅脑MRI检查急性期均显示以大脑后部为主的异常信号,恢复期异常信号可明显减少或消失。结论 RPLS临床表现为脑病的症状,急性期MRI的脑白质异常是可逆的。     

Clinical spectrum of reversible posterior leukoencephalopathy syndrome

BACKGROUND: Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by neuroimaging findings of reversible vasogenic subcortical edema without infarction. The clinical syndrome of RPLS typically involves headache, encephalopathy, visual symptoms, and seizures. OBJECTIVE: To retrospectively identify patients with RPLS with a characteristic clinical presentation and neuroimaging abnormalities and documented improvement on repeated neuroimaging. DESIGN: Retrospective. SETTING: Mayo Clinic. PATIENTS: Thirty-six patients with RPLS. MAIN OUTCOME MEASURES: Associated comorbid medical conditions, presenting clinical symptoms, duration of clinical symptoms, diagnostic test results (magnetic resonance imaging, electroencephalography, and lumbar puncture), and time to clinical and neuroimaging recovery. RESULTS: We identified 38 episodes of RPLS in 36 patients (20 females and 16 males) with a mean age of 44.7 years. Comorbid conditions included hypertension (53%), renal disease (45%), dialysis dependency (21%), malignancy (32%), and transplantation (24%). Presenting symptoms included clinical seizures (87%), encephalopathy (92%), visual symptoms (39%), and headache (53%). Mean peak systolic blood pressure at presentation was 187 mm Hg. Clinical symptoms resolved after a mean of 5.3 days. Atypical neuroimaging features included significant frontal involvement in 22 episodes (58%), gray matter lesions in 16 (42%), unilateral lesions in 2 (5%), hemorrhage in 2 (5%), recurrent RPLS in 2 (5%), confluent lesions in 2 (5%), and foci of permanent injury in 10 (26%). Twenty-two episodes (58%) had brainstem/cerebellar involvement on neuroimaging. CONCLUSIONS: This is the largest clinical series to date of RPLS with confirmed neuroimaging improvement. Clinical recovery occurred in most patients within days. The condition was rarely isolated to the parieto-occipital white matter, and atypical neuroimaging features were frequent.     

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.     

Dialysis disequilibrium: another reversible posterior leukoencephalopathy syndrome?

Dialysis disequilibrium syndrome is a disorder of the central nervous system in patients on dialysis. The underlying etiology is thought to be primarily due to cerebral edema; however, neuroradiologic findings have not been described previously. We describe a patient who presented with new onset headaches and status epilepticus after beginning hemodialysis. Her neuroimaging studies revealed white matter changes in the posterior parietal and occipital lobes similar to those seen in patients with reversible posterior leukoencephalopathy syndrome (RPLS). This case suggests that dialysis disequilibrium syndrome and RPLS may represent a spectrum of disorders in which the underlying mechanism is vasogenic edema.     

可逆性后部白质脑病综合征8例临床分析

目的 探讨可逆性后部白质脑病综合征(RPLS)临床和影像学特点. 方法回顾性分析8例RPLS患者临床及影像学资料.结果 本组患者继发于高血压2例,子痫2例,产褥期子痫1例,血栓性血小板减少性紫癜1例,肺性脑病1例,颅咽管瘤1例.临床表现:6例出现头晕,4例出现癫NFDCC发作,3例出现头痛, 2例出现视物模糊,恶心、呕吐、精神异常、脑疝、左侧轻偏瘫各1例.6例无神经系统定位体征.影像学检查颅脑CT检查 1例脑干低密度影,4例出现枕叶低密度影(其中2例广泛脑白质水肿),1例未发现异常.7例颅脑MRI检查(T1、T2、FLAIR),6例均有枕叶受累,3例同时累及额颞叶,1例累及脑干和小脑,1例累及尾状核头和丘脑,在脑叶呈脑回样,在其他部位呈斑片样异常信号;T1WI呈略低或等信号,T2WI呈高信号,FLAIR像显示皮质和皮质下白质明显高信号.结论 头晕、头痛、视觉障碍和癫NFDCC发作是RPLS主要临床表现,影像学特征主要为大脑后部白质对称性长T1 、长T2 信号.     

12例可逆性后部白质脑病综合征的临床特点

目的探讨可逆性后部白质脑病综合征(RPLS)的临床特点。方法回顾性分析2015-06—2016-09于河南省人民医院诊治的12例可逆性后部白质脑病综合征患者的临床特征、影像学特点、实验室检查并复习相关文献。结果临床特征:本组女11例,男1例。子痫10例,合并血小板减少7例;原发性高血压2例。10例出现癫痫发作,7例出现头痛,6例视物障碍。影像学特点:病灶大多为对称性,主要位于双侧大脑后部白质,少数位于额顶叶、基底节区、胼胝体、小脑及其他部位。在CT呈低密度影,MRI呈长T_1、长T_2,FLAIR呈高信号,DWI大部分呈低信号,ADC大部分呈高信号。4例复查后病灶完全恢复,呈完全可逆性。实验室检查:LDH升高11例,其中血小板减少伴白细胞升高、肌酸激酶升高7例。结论 RPLS是一种女性好发,以头痛、癫痫发作、视觉障碍、意识障碍为主要表现,影像学表现为可逆的血管源性水肿的神经系统疾病,血小板减少可能为其病因之一,经积极治疗,大部分预后良好。     

Reversible posterior leucoencephalopathy syndrome associated with bone marrow transplantation

Reversible posterior leucoencephalopathy syndrome (RPLS) has previously been described in patients who have renal insufficiency, eclampsia, hypertensive encephalopathy and patients receiving immunosuppressive therapy. The mechanism by which immunosuppressive agents can cause this syndrome is not clear, but it is probably related with cytotoxic effects of these agents on the vascular endothelium. We report eight patients who received cyclosporine A (CSA) after allogeneic bone marrow transplantation or as treatment for severe aplastic anemia (SSA) who developed posterior leucoencephalopathy. The most common signs and symptoms were seizures and headache. Neurological dysfunction occurred preceded by or concomitant with high blood pressure and some degree of acute renal failure in six patients. Computerized tomography studies showed low-density white matter lesions involving the posterior areas of cerebral hemispheres. Symptoms and neuroimaging abnormalities were reversible and improvement occurred in all patients when given lower doses of CSA or when the drug was withdrawn. RPLS may be considered an expression of CSA neurotoxicity.     

38例可逆性后部白质脑病综合征的临床影像学特征及病因学分析

目的探讨可逆性后部白质脑病综合征(RPLS)的临床影像学特点以及病因学。方法回顾性分析2009年至2014年间在中南大学湘雅医院神经内科就诊的38例病人临床资料、影像学资料及治疗过程。结果 38例患者中女性23例,男性15例;高血压病史31例,有子痫或子痫前期病史6例;服用免疫抑制剂5例,肿瘤化疗史3例。30例(78.9%)患者首发表现为头痛头晕,此外血压增高、恶心呕吐、视力下降、痫性发作和意识障碍为主要临床表现。磁共振影像表现为大脑后半部对称性稍长T1、长T2信号,FLAIR序列为高信号,DWI低信号,并可发现皮质受累。结论 RPLS是一类预后良好的脑病综合征,病因复杂,目前主要依靠临床表现以及影像学明确诊断。