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。     

脑后部可逆性脑病综合征MRI及DWI表现及临床意义

目的分析脑后部可逆性脑病MRI及DWI的影像学特点。方法 15例经临床诊治及MRI检查的脑后可逆性脑病综合征(Poster revers Tble encephalopathy.syndrome,PRES)患者的资料,均常规MRI及DWI扫描并计算出ADC图。结果 15例双侧大脑半球后部皮层受损,以枕叶、顶叶、颞叶好发,其中11例伴额叶受损,3例伴侧脑室深部白质、基底节受损,2例伴丘脑、小脑半球受损。13例发病时以血管源性水肿为主,2例为细胞毒性水肿。5例在首次检查后7~21d复查,3例病灶为可逆性完全消失,2例大部分病灶可逆性消失,局部有残留病灶。结论脑后部可逆性脑病综合征影像学具有特点,DWI、ADC容易区分血管源性水肿和细胞毒性水肿病灶,有助于临床鉴别及判断预后。     

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。     

可逆性脑后部白质病变综合征影像学特点分析

目的探讨可逆性脑后部白质病变综合征的影像学特点。方法回顾性分析11例可逆性脑后部白质病变综合征患者的影像学资料。结果患者中累及枕叶9例,顶叶8例,额叶8例,基底节6例,颞叶2例,胼胝体1例和小脑1例。CT平扫示枕叶、顶叶、额叶、基底节、颞叶、胼胝体和小脑等低密度。MRI平扫示T_1WI为低信号、T_2WI及Flair为高信号。DWI可呈等或稍高信号,ADC可呈等、稍高、高或低信号。结论可逆性脑后部白质病变综合征可累及部位主要局限于顶枕叶,但额叶、基底节、颞叶、胼胝体及小脑等部位亦可受累。Flair、DWI和ADC序列对病变最为敏感。     

重度子痫前期与子痫并发可逆性后部白质脑病综合征的临床特征分析

目的探讨重度子痫前期与子痫并发可逆性后部白质脑病综合征(RPLS)的临床特征。方法回顾性分析32例重度子痫前期与子痫并发RPLS患者的临床资料。结果 32例患者均表现为迅速进展的血压升高、头痛;意识障碍、精神障碍及行为异常25例;痫性发作23例;视觉障碍19例;影像学显示大脑后部枕叶及顶叶为主的白质水肿;经及时治疗患者的临床症状及影像学改变迅速恢复。结论重度子痫前期与子痫并发RPLS患者具有特征性的临床及影像学改变,早期诊断及治疗,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.     

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。     

妊娠相关性脑静脉及静脉窦血栓形成和可逆性后部白质脑病的磁共振鉴别

目的探讨MRI和MRV在妊娠相关性脑静脉及静脉窦血栓形成(CVST)和可逆性后部白质脑病综合征(RPLS)鉴别诊断中的价值。方法回顾性分析3例妊娠相关性CVST和6例重度子痫前期、子痫发生RPLS患者的影像学资料。所有患者均行MRI和DSA检查,其中7例患者行MRV检查。结果 3例CVST患者中,1例孤立性大脑上静脉前组血栓形成,MRI表现为相应部位T1WI低、等信号,T2WI、FLAIR等、稍高信号,DWI为等、高信号,ADC图为低、稍高信号;2例横窦血栓形成,表现为双侧枕叶皮质、皮质下白质片状T1WI低信号,T2WI、FLAIR高信号,DWI、ADC高信号,可不对称性地累及顶叶、小脑半球,MRV与DSA检查结果相符。6例RPLS患者MRI显示双侧顶、枕叶皮质、皮质下白质多发性、斑片状、对称性病变,部分严重患者大脑半球呈弥漫性、大片状受累,表现为T1WI低信号,T2WI、FLAIR高信号,DWI、ADC高信号;1例患者MRV检查示左侧横窦未显影,DSA检查示左侧横窦通畅。结论横窦血栓形成和RPLS患者MRI均显示血管源性脑水肿,两者脑水肿主要发生于双侧枕、顶叶,但前者范围较局限,后者范围更广泛,可累及基底节、额叶、颞叶。RPLS患者MRV检查可有假阳性,DSA是鉴别两者的重要检查方法。     

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。     

Acute cortical blindness due to posterior reversible encephalopathy

An acutely hypertensive 55 year-old male experienced seizures and cortical blindness post-operatively. CT scans demonstrated hypointensities in the occipital lobes bilaterally. MRI revealed symmetrical bilateral hyperintense signals in the same region, involving both grey and white matter. Thromboembolic screening investigations including vertebral artery doppler studies were normal and echocardiography demonstrated borderline left ventricular hypertrophy. A diagnosis of posterior reversible encephalopathy syndrome (PRES) was reached and there was complete resolution of blindness with antihypertensive therapy. This case supports the vasogenic theory of PRES which suggests that sustained high grade fluctuations in blood pressure lead to a reduction in cerebral vascular autoregulatory function. The resultant failure of compensatory vasoconstriction to prevent hyperperfusion causes fluid to extravasate into the occipital lobes, which in the present case resulted in cortical blindness.     

Asymmetric reversible posterior leukoencephalopathy syndrome

Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized radiographically by magnetic resonance imaging as white matter hyperintensities, which reflect cerebral edema. These changes are typically restricted to the parietal and occipital lobes, and are usually quite symmetric. We report a case of asymmetric RPLS involving only one frontal lobe in a patient with severe hypertension chronic internal carotid artery stenosis, and ipsilateral vasogenic edema.     

Four cases of reversible posterior leukoencephalopathy syndrome

We reported four children cases with reversible posterior leukoencephalopathy syndrome (RPLS). Magnetic resonance imaging (MRI) of the brain demonstrated reversible multiple cortical and subcortical lesions predominant in the occipital region. All patients presented with neurological symptoms associated with hypertension, such as headache, seizures and visual disturbances, which were successfully treated with antihypertensive therapy. Although RPLS is rare in childhood, characteristic lesions on MRI in the hypertensive children should be recognized as manifestations of RPLS. Subsequent clinical management should focus on the treatment of the hypertension and/or its underlying causes.     

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.     

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。     

可复性后部脑病综合征的影像学诊断

目的探讨可复性后部脑病综合征（PRES）的影像学表现.方法回顾性分析了12例PRES病人的临床和影像学资料,其中9例为子痫/先兆子痫,2例为高血压脑病,1例为环孢菌素A（CSA）的神经毒性.12例均行MRI检查,其中7例同时行钆喷替酸葡甲胺（Gd-DTPA）增强扫描,4例行磁共振血管造影（3D-TOF MRA）检查,1例行弥散加权成像（DWI）.7例行CT平扫检查,2例行脑血管造影（DSA）检查.结果MRI显示病灶基本上呈双侧对称性分布,多数病灶位于顶、枕叶脑实质内,T1WI呈等或略低信号,T2WI呈高信号,FLAIR像显示皮层和皮层下白质明显高信号影,较T1WI、T2WI更加清楚.注射Gd-DTPA后多无明显异常对比增强.1例DWI显示双侧顶、枕叶及额叶皮层内弥散受限呈高信号,ADC图显示邻近的皮层下白质呈高信号.4例CT显示双侧顶、枕叶及额叶对称性斑片状低密度影,3例CT未见异常.经对症处理后复查示所有病灶几乎完全吸收消失.结论PRES的影像学表现具有特征性.MRI应作为诊断本病的首选手段.     

Multiple cavitations in posterior reversible leukoencephalopathy syndrome associated with hemolytic-uremic syndrome

We describe a 4-year-old boy with posterior reversible leukoencephalopathy syndrome associated with hemolytic-uremic syndrome. He exhibited bloody stool by Escherichia coli O157: H7 infection with acute renal failure. He subsequently presented high blood pressure, followed by visual disturbance and loss of consciousness. Brain MRI revealed bilateral occipital high intensities by T2-weighted images and high value by apparent diffusion coefficient map, thus we made a diagnosis of posterior reversible leukoencephaly syndrome associated with hemolytic-uremic syndrome. In spite of immediate blood pressure control, occipital lesions developed day by day, resulting in multiple subcortical cavitations. Although posterior reversible leukoencephalopathy syndrome is originally characterized by reversible vasogenic edema, this case rarely resulted in irreversible changes with cystic formation. We concluded that precipitating factors, i.e., clotting abnormalities, Shiga toxin, vasospasms and endothelial dysfunction might have synergistically induced irreversible brain infarcts, and caused unusual cavitations.     

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。     

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.     

Case of posterior reversible encephalopathy syndrome with cerebral vasoconstriction]

A 56-year-old woman attended our hospital because of acute severe (thunderclap) headache. Neurological examination was normal and no abnormality was found on head CT or by cerebrospinal fluid examination. A few days later, she experienced a recurrence and suffered a seizure in her left upper and lower extremities. On neurological examination, she had conjugate deviation of the eyes toward the right side and left lower limb paralysis with Chaddock sign. MRI showed multiple hyperintense lesions in the bilateral occipital and parietal lobes, predominantly in the subcortical white matter at the right side on T2-weighted and FLAIR images. We diagnosed posterior reversible encephalopathy syndrome (PRES) because the ADC map showed a vasogenic edema pattern (increased ADC values in the hypodense lesions on diffusion-weighted image). Her blood pressure was normal and there were no underling diseases. As MRA showed vasoconstriction especially in bilateral posterior cerebral arteries, we initiated a therapy with a Ca-channel blocker. On follow-up MRI, the hyperintense lesions on T2-weighted and FLAIR images had almost disappeared, and vasoconstriction was also improved on MRA. This case suggested that cerebral vasoconstriction could underlie both thunderclap headache and PRES.