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。     

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.     

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。     

酷似中毒性脑病的可逆性后部白质脑病综合征:6例临床分析

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,为与中毒性脑病进行鉴别提供依据。方法回顾性分析6例RPLS患者的临床特点、影像学资料及治疗经过。结果 6例患者分别有大量饮酒史、海洛因吸食史、煤气中毒史、长期接触油漆涂料史、一氧化碳吸入史和有机溶剂接触史。临床主要表现为头痛、恶心、呕吐、癫痫发作以及血压升高。头部MRI主要表现为双侧颞叶、顶叶、额叶、侧脑室旁白质、小脑、脑干病灶,呈长T1、长T2信号,无强化。6例患者对症及去除病因治疗后临床表现及影像学表现均明显好转。结论 RPLS患者可以出现与中毒性脑病相似的临床及影像学特点,特别是当患者有毒物吸入史时,应根据该病相对特征性的临床、影像学及疗效尽早排除。     

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。     

重度子癎前期发生可逆性后部白质脑病综合征3例（附文献复习）

目的：探讨重度子癎前期发生可逆性后部白质脑病综合征（RPLS）的临床表现及治疗原则。方法：对3例重度子癎前期发生RPLS患者的临床症状、体征、实验室和影像学检查、治疗和预后资料结合6例文献报道进行分析。结果：患者均急性发病,常表现为头痛、视朦和皮质盲。平均动脉压急性升高至130mmHg（1mmHg=0.133kPa）左右时发生RPLS,病灶以枕、顶叶为主。9例患者中4例并发HELLP综合征。经积极治疗,患者症状、体征迅速改善,预后良好。结论：①颅内高压和皮质盲是重度子癎前期发生RPLS时的主要临床表现;②重度子癎前期患者脑血管自动调节机制的上限血压下移,血管内皮功能受损明显;③及时终止妊娠和积极降压、脱水是治疗成功的关键。     

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.     

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.     

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.     

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。