可逆性后部白质脑病综合征临床特点及影像学改变--附12例临床分析

目的：探讨可逆性后部白质脑病综合征（PRES ）的临床特点和影像学改变。方法回顾性分析12例PRES患者的临床和影像学资料。结果原发病为慢性酒精中毒1例，系统性红斑狼疮伴特发性血小板减少性紫癜1例，余10例均与妊娠相关，其中7例原发病为妊娠高血压综合征（妊高征）。临床主要表现为癫痫发作（9例）、视力障碍（5例）、意识障碍（9例）、头痛（7例）、恶心呕吐（3例）；10例患者存在肾功能损害。颅脑C T 及M R检查示病灶累及顶枕叶（10例）、额叶（3例）、基底节（3例）、小脑（2例）和脑干（2例），M RI均呈长T1长T2信号，T2 flair高信号，DWI等信号（7例）或略高信号（1例）；颅脑CT 呈低密度（8例）或等密度（2例）；边界欠清。经积极对因对症治疗，本组患者症状在中位数4d（QL3.25d，QU5d）后几乎完全缓解，影像学表现在中位数9 d（QL 7.25 d ，QU 10.75 d）后得到完全或大部分恢复。结论 PRES以癫痫发作、视力障碍、意识障碍、头痛、恶心呕吐为主要临床表现，影像学改变以可逆的、多分布于后循环供血区的皮层下脑白质血管源性水肿为特点， MRI呈T1低信号、T2及T2 flair高信号，DWI等/略高信号，CT呈低/等密度。积极治疗后多数患者症状可完全缓解，影像学表现可完全或大部分恢复。     

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.     

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（PRES）的临床和影像学特点。方法 回顾性分析7例PRES患者临床及影像学资料。结果 本组继发于妊娠高血压综合征3例，肾功能衰竭3例，高血压1例。临床表现：7例均有头痛及视物模糊，伴有癫痫发作6例，恶心、呕吐4例，轻偏瘫、共济失调各1例。6例行头颅CT检查，3例枕叶低密度影，其中2例广泛脑白质水肿；1例多发小血肿；2例未发现异常。7例MRI检查显示枕叶均受累，同时伴小脑受累3例，脑干2例，额顶叶皮质下白质2例，尾状核、丘脑各1例。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和Fair像呈高信号。2例增强扫描1例无强化，1例呈脑回样、斑片样和环状强化。4例DWI扫描，2例呈略高信号，1例呈低信号，1例未见异常。结论 头痛、视觉障碍和癫痫发作是PRES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。     

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。     

获得性肝性脑部变性的临床与影像学特点

目的探讨获得性肝性脑部变性的临床与影像学特点。方法回顾性分析5例确诊患者的临床资料,总结其临床表现、影像学检查、临床疗效及预后。结果5例患者临床表现均有认知能力下降,帕金森病综合征2例,构音不清2例,精神异常、舞蹈病和共济失调各1例。头颅MRI检查5例双侧苍白球对称性T1WI高信号,2例中脑红核对称性T1WI高信号。治疗后1例病人有好转,3例病人无明显变化,1例病人病情恶化。结论获得性肝性脑部变性是一种发病机制不完全清楚且治疗困难的临床综合征,其特异性的MRI表现有助于诊断。     

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。     

Marchiafava-Bignami病的临床及影像学研究

目的 探讨Marchiafava-Bignami病(MBD)的临床及影像学改变.方法 回顾性分析了7例MBD患者的临床和CT、MRI资料,包括病灶形态、分布、信号或密度改变等影像学特征:4例同时行CT和MRI检查,2例仅行CT检查,1例仅行MRJ检查.结果 本组患者急性型5例,均表现为胼胝体肿胀及长T1、长T2信号改变,均有双侧脑室周围白质、额叶皮层下白质对称性累及:慢性型2例,胼胝体明显萎缩变薄,并呈长T1、长T2信号及FLAIR像点片状或线样低信号灶.5例患者DWI显示病灶区信号明显增高并有2例出现弥散受限改变.结论 MBD具有特征性MRJ表现,其影像学改变可能反映其临床及预后.     

并发颅内出血的可逆性后部脑病综合征5例临床分析并文献复习

目的探讨可逆性后部脑病综合征(posterior reversible encephalopathy syndrome,PRES)并发颅内出血的临床及影像学特点。方法回顾性分析我院5例并发颅内出血的PRES患者的临床及影像学特点。结果 5例PRES患者均为年轻女性,4例继发于子痫前期,另1例继发于慢性肾衰竭。临床表现为头痛、癫痫发作、视物模糊、意识状态改变及局灶性神经功能缺损等。影像主要表现为双侧大脑后部为主的血管源性脑水肿,但也有额叶、基底节、脑干、小脑受累和细胞毒性脑水肿等不典型表现。5例患者均合并脑实质血肿,4例位于水肿周围,1例并发硬膜下血肿。5例患者均合并血小板减少及凝血功能异常,且均因病情危重而入住重症监护病房,经积极治疗后临床表现及影像学表现均明显好转。结论合并凝血功能异常的PRES较易出现颅内出血等并发症。     

胼胝体压部可逆性病变的MRI诊断与临床分析

目的探讨胼胝体压部(splenium of the corpus callosum,SCC)可逆性病变的MRI表现及临床特点。方法回顾性分析8例脑MRI表现为单纯胼胝体压部可逆性病变患者的临床和影像学资料。结果 8例患者的SCC可逆性病变均为继发性,原发病分别为脑内感染5例,肝豆状核变性、低血糖脑病及脑外伤各1例。8例患者均急性起病,临床表现为发热、头痛5例,急性意识障碍2例,肌张力增高2例,颈强2例,均符合原发病的临床表现。8例均行脑MRI检查,均表现为T1低或等信号,T2及FLAIR序列高信号,DWI高信号(提示细胞源性水肿),其中4例行增强扫描未见强化。8例患者均于临床症状好转或消失后复查MRI提示病灶消失。结论 SCC可逆性病变在多种疾病中均可出现,无该病变相关的特异性临床表现,MRI表现提示病灶为细胞源性水肿可能。     

表现为家族性偏头痛的CADASIL家系的影像学特点

目的探讨伴皮层下梗死和白质脑病的常染色体显性遗传性脑病(CADASIL)家系的核磁共振(MRI)特点,提高对本病脑内MRI表现的认识。方法记录该CADASIL家系先证者及其亲属的临床表现及影像学检查,并行NOTCH3基因检测。结果本组6例中5例检出NOTCH3基因第14外显子C2182T突变,其中3例与MRI初诊结果一致。头颅核磁共振显示双侧额叶皮层下与侧脑室旁白质内广泛融合的病灶,基本对称性分布的稍长T1T2信号,T2FLAIR呈高信号者4例;外囊T2WI呈高信号3例;基底节区、丘脑腔隙性梗死4例;脑干T2WI呈高信号2例;脑内微出血3例;未见双侧颞极白质病灶(O’Sullivin征)。结论 CADASIL病例MRI存在特征性的病变,MRI对该病的诊断有重要作用。     

Status epilepticus as initial manifestation of posterior reversible encephalopathy syndrome

We report 10 cases of status epilepticus (SE) in patients with posterior reversible encephalopathy syndrome (PRES). In all cases, SE brought PRES to medical attention. The majority of the cases had focal-onset complex partial SE. Complete resolution of SE was achieved after combined treatment of PRES and SE in all cases. SE in the setting of PRES carries a favorable prognosis but requires timely recognition and treatment of the course of PRES.     

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.     

Long-term Neurocognitive and Neuroimaging Outcomes in Posterior Reversible Encephalopathy Syndrome: Two Case Reports and Implications

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic and neurometabolic state associated with a distinct appearance on computed tomography and magnetic resonance imaging, as well as particular clinical features. While PRES is defined as a reversible condition, instances of irreversible or persisting cytotoxic edema have been reported and appear to be associated with poor outcomes. Studies addressing the post-acute neurocognitive findings in persisting/irreversible PRES are lacking. We report two cases of persisting PRES with associated neurocognitive deficits and functional limitations following acute presentation. Neuroimaging revealed abnormalities within the posterior parietal lobes, along with bilateral involvement of the occipital and frontal lobes. The results from the neuropsychological examination revealed a pattern of impairment generally affecting spatial-perceptual and related functions and aspects of executive function. These cases document instances of irreversible/persisting PRES with associated neurocognitive deficits and functional limitations generally corresponding with the areas implicated on neuroimaging. Studies addressing the post-acute neurocognitive presentation and functional outcomes of PRES are needed.     

Long-term neurocognitive and neuroimaging outcomes in posterior reversible encephalopathy syndrome: two case reports and implications

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic and neurometabolic state associated with a distinct appearance on computed tomography and magnetic resonance imaging, as well as particular clinical features. While PRES is defined as a reversible condition, instances of irreversible or persisting cytotoxic edema have been reported and appear to be associated with poor outcomes. Studies addressing the post-acute neurocognitive findings in persisting/irreversible PRES are lacking. We report two cases of persisting PRES with associated neurocognitive deficits and functional limitations following acute presentation. Neuroimaging revealed abnormalities within the posterior parietal lobes, along with bilateral involvement of the occipital and frontal lobes. The results from the neuropsychological examination revealed a pattern of impairment generally affecting spatial-perceptual and related functions and aspects of executive function. These cases document instances of irreversible/persisting PRES with associated neurocognitive deficits and functional limitations generally corresponding with the areas implicated on neuroimaging. Studies addressing the post-acute neurocognitive presentation and functional outcomes of PRES are needed.     

Posterior reversible encephalopathy syndrome (PRES): a rare condition after resection of posterior fossa tumors: two new cases and review of the literature

Introduction

In 1996, Hinchey and colleagues coined the term “Posterior reversible encephalopathy syndrome” (PRES) to describe a condition seen in patients with acute neurological symptoms and reversible subcortical vasogenic edema predominantly involving parieto-occipital areas demonstrated in brain MRI. The occurrence of this phenomenon after surgical resection of CNS tumors is typically linked to pediatric cases.

Material and Methods

Two new cases of PRES after posterior fossa surgery are reported. A thorough review of the literature is carried out with the purpose of updating and summarizing the main features regarding PRES in similar cases. Seven cases ofPRES after resection of a posterior fossa tumor have been hitherto reported (4 patients were <20 years old). Thereis another pediatric case described after a ventriculoperitoneal shunting procedure in a patient with fourth ventricleependymoma. Two resected tumors were ependymomas, 2 hemangiopericytomas in one patient, 1 pilocyticastrocytoma, 1 vestibular schwannoma, and 1 of the reported cases did not describe the final pathology diagnosis.

Case Reports

We present 2 new cases of PRES after surgical resection of a posterior fossa tumor (medulloblastoma in case 1and ependymoma in case 2) in pediatric patients. Case 1 developed delayed seizures and altered mental status(10 days after surgical resection) after receiving treatment with bromocriptine for cerebellar mutism. Case 2presented with generalized seizures and altered mental status within the first 48 postoperative hours followed byright hemiparesis. Both patients fully recovered and returned to neurological baseline status. A thorough review ofthe literature was carried out with the purpose of updating and summarizing the main features regarding PRES insimilar cases.

Conclusions

We report 2 new pediatric cases of posterior reversible encephalopathy syndrome (PRES) that developed after surgical resection of a posterior fossa tumor. Appropriate management includes supportive measures, antihypertensive agents, and antiepileptic drugs, if needed. Full recovery is the most likely outcome in line with previous articles.

     

Elevation of serum lactate dehydrogenase at posterior reversible encephalopathy syndrome onset in chemotherapy-treated cancer patients

The pathophysiology of posterior reversible encephalopathy syndrome (PRES) is incompletely understood; however, an underlying state of immune dysregulation and endothelial dysfunction has been proposed. We examined alterations of serum lactate dehydrogenase (LDH), a marker of endothelial dysfunction, relative to the development of PRES in patients receiving chemotherapy. A retrospective Institutional Review Board approved database of 88 PRES patients was examined. PRES diagnosis was confirmed by congruent clinical diagnosis and MRI. Clinical features at presentation were recorded. Serum LDH values were collected at three time points: prior to, at the time of, and following PRES diagnosis. Student’s t-test was employed. LDH values were available during the course of treatment in 12 patients (nine women; mean age 57.8 years [range 33–75 years]). Chemotherapy-associated PRES patients were more likely to be normotensive (25%) versus the non-chemotherapy group (9%). LDH levels at the time of PRES diagnosis were higher than those before and after (p = 0.0263), with a mean difference of 114.8 international units/L. Mean time intervals between LDH measurement prior to and following PRES diagnosis were 44.8 days and 51.4 days, respectively. Mean elapsed time between last chemotherapy administration and PRES onset was 11.1 days. In conclusion, serum LDH, a marker of endothelial dysfunction, shows statistically significant elevation at the onset of PRES toxicity in cancer patients receiving chemotherapy. Our findings support a systemic process characterized by endothelial injury/dysfunction as a factor, if not the prime event, in the pathophysiology of PRES.     

Posterior reversible encephalopathy syndrome as a complication of acute lupus activity

Objectives

We aimed to describe the clinical and imaging characteristics; associated risk factors and neurological outcome of posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE).

Methods

From October 2001 to January 2007, we identified patients with SLE and the criteria for PRES in our institution, which is a tertiary-care referral center for patients with SLE; the patients were evaluated at baseline and followed to determine the clinical outcome.

Results

We identified 22 episodes of PRES in 21 patients; 20 (95.2%) were women, mean age of onset was 24.9 ± 8.6 years, all patients had high systemic activity (SLEDAI scores from 12 to 39). Acute hypertension was observed in 18 episodes (81.8%), and renal failure in 16 (72.7%); only 3 patients were on cyclophosphamide at the time of the onset of PRES. Persistent neurological deficit was observed in 2 cases; one patient died during the acute episode.

Conclusions

PRES is a central nervous system syndrome that is observed in SLE patients. It was associated mainly to high systemic activity, acute hypertension, and renal failure. Although reversibility is common, residual neurological damage may be observed.     

Isolated brainstem involvement in posterior reversible encephalopathy syndrome: a case report and review of the literature

Posterior reversible encephalopathy syndrome (PRES) is a clinical–radiological syndrome, reversible vasogenic edema predominantly within parieto-occipital regions. However, isolated brainstem involvement in PRES has been rarely reported, little is known about its clinical manifestations, radiological features and outcomes. We reported a case with PRES with only brainstem involvement and performed a systematic review of published cases. Twenty-four cases, together with our case, were included in the analysis. Mean age was 43?years and 63% were males. Hypertension (50%), nephropathy (25%) and chemotherapy (21%) were the major risk factors. All patients except two had acute hypertension and 50% of patients had renal dysfunction at onset. The most common symptoms were altered consciousness (46%) and headache (46%), Seizure was only presented in 21% of patients. All patients except two were treated with antihypertension. Most patients recovered to their neurological baseline. Clinicians should recognize this unique variant finding in PRES. which always affects males with severe hypertension, especially combined with renal dysfunction. Antihypertensive treatment is the most widely used therapy. Outcome is usually well.     

Posterior reversible encephalopathy syndrome following hemodynamic treatment of aneurysmal subarachnoid hemorrhage-induced vasospasm

Posterior reversible encephalopathy syndrome (PRES) is an uncommon but significant complication of hemodynamic therapy after aneurysmal subarachnoid hemorrhage (aSAH)-induced vasospasm. We performed a PubMed literature search for the period January 1999 to January 2015 using the search terms “posterior reversible encephalopathy syndrome”, “subarachnoid hemorrhage”, “vasospasm”, and “hypertensive encephalopathy”, and identified nine cases of PRES after aSAH-induced vasospasm in the literature. We also present a 63-year-old man with aSAH complicated by vasospasm treated with hemodynamic augmentation who subsequently developed PRES. Imaging following development of PRES symptoms shows vasogenic edema in the white matter of the parietal and occipital lobes. Age, sex, history of hypertension, and baseline blood pressure were variable among patients in the literature review. In all cases, patients improved both from a radiological and clinical perspective following blood pressure reduction. To summarize, PRES is a rare complication of hemodynamic therapy for vasospasm following aSAH. The literature at the time of writing demonstrates no common pattern with regard to patient demographics, medical history, or mode of treatment for symptomatic vasospasm. Given its sporadic and unpredictable nature, considering PRES in the differential diagnosis is important when addressing neurological decline following hemodynamic treatment of vasospasm related to aSAH.