可逆性后部白质脑病综合征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62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21例(33.9%),高血压病19例(30.8%),肾功能不全11例(17.7%),其他原因为红斑狼疮、应用化疗药物、细胞毒性药物、血管炎、肾病综合征、急性肾小球肾炎、肾动脉狭窄、输血。头痛、癫痫发作、意识障碍、视力障碍是RPLS常见四联症,59例伴急性血压增高。影像学表现为大脑后部对称性白质水肿,CT低密度灶,MRI长T1低信号,短T2高信号,Flair像高信号。57例累及双侧顶枕叶,5例累及单侧枕叶,其他部位受累频率从高依次为:额叶、颞叶、小脑、基底核区、脑干、丘脑、胼胝体。经过降压、抗癫痫、减轻脑水肿对因、对症治疗,60例患者临床症状、影像学及临床症状异常迅速恢复。结论头痛、癫痫发作、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。大多数患者经治疗迅速恢复。     

子痫并发可逆性后部白质脑病一例

可逆性后部白质脑病(RPLS)是一种神经影像学综合征,本文回顾性分析1例并发RPLS的子痫患者的临床资料,其临床表现为头痛、精神改变、痫性发作、视觉改变,颅脑核磁共振示:双侧额、顶叶及双侧基底核区异常信号,治疗11d后复查核磁恢复正常。     

老年人可逆性后部白质脑病综合征五例及临床和影像学特点分析

目的 分析我国老年人可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)患者的临床表现和影像学特点.方法 回顾报告5例老年RPLS患者的临床表现和影像学特点. 结果 本组老年男性患者1例,女性4例,平均年龄(63.0±2.4)岁,5例患者均在起病时并存高血压.最常见的临床表现依次为视觉障碍5例,头痛4例,癫痫4例,意识障碍3例,病理反射2例,偏瘫2例.CT检查示4例患者表现异常,MRI检查示5例患者均出现枕叶病变,颞叶受累2例,顶叶受累1例.4例患者出院平均(48.8±29.2)d后CT检查为阴性结果,死亡1例.结论 RPLS是典型的多因共果疾病,血管原性假说被影像学研究结果证实.神经影像学对诊断RPLS很重要,弥散加权像(DWI)有助于鉴别诊断RPLS和缺血性脑血管病.     

可逆性后部白质脑病综合征的发病机制

可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)是一种临床影像学疾病实体,主要以迅速进展的血压增高、头痛、呕吐、意识障碍、痢性发作为特征,神经影像学显示为双侧大脑半球对称性白质可逆性水肿(尤其是在大脑后部),通过及时和正确的治疗,临床症状和神经影像学改变可完全恢复.有关RPLS发病机制的两大假说--脑血管痉挛学说和脑血管过度灌注学说一直存在争论,目前多数学者仍然认同后者是造成脑水肿的主要原因.文章从RPLS的病因学、病理学和影像学特征阐述了其发病机制以及近年来对上述两大假说的新观点.     

不典型可逆性后部白质脑病综合征2例报告

可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)是一组以头痛、恶心、呕吐、癫痫发作,视力障碍,意识和精神改变为主要临床表现的综合征.影像学检查病灶主要位于顶枕叶白质,病程经过以可逆性为特征.现就我院诊治、资料完整的2例不典型RPLS报告如下.     

可逆性后部白质脑病综合征

<正>可逆性后部白质脑病综合征(RPLS)是一组多病因、多症状的临床综合征,为急性或亚急性起病,主要临床表现为头痛、癫痫发作、视觉障碍、意识障碍、精神异常等,影像学上以大脑后部白质改变为主,预后一般较好,绝大多数患者神经系统损伤症状能够完全恢复,但治疗不及时,也可造成脑组织不可逆性损伤。本文就RPLS研究现状予以综述。1命名RPLS的概念最早在1996年由Hinchey等[1]提     

妊娠相关可逆性后部白质脑病综合征的临床与MRI表现3例报告

可逆性后部白质脑病综合征（reversible posterior leukoencephalopathy syndrome,RPLS）的概念最早于1996年由Judy Hinchey提出,是由恶性高血压、子痫、严重肾脏疾病、某些已被证实与RPLS相关的免疫抑制剂和化疗药物等引起的临床综合征。现将我科收治的3例妊娠相关RPLS的临床表现和影像学特征报道如下,以提高对该病的认识。     

可逆性后部白质脑病综合征9例临床分析

目的总结可逆性后部白质脑病综合征(RPLS)的临床特征,提高本病的诊断水平。方法对确诊的9例RPLS患者临床表现、颅脑CT及MRI检查结果进行分析。结果急性起病7例,亚急性起病2例。临床表现为头痛7例,视觉障碍6例,癫痫发作5例,意识障碍6例。CT检查病灶表现为低密度影,MRI呈稍长或等T1、长T2信号,Flair像为高信号、DWI为低或等信号、ADC为高信号;其中累及枕叶6例,顶叶6例,额叶3例,颞叶1例,小脑1例,脑干1例,侧脑室3例,基底节3例,丘脑1例,海马1例;9例均累及白质,其中4例累及灰质。结论 RPLS的临床特征是急性或亚急性起病,以头痛、癫痫、意识障碍、视力障碍为主要表现。RPLS影像学表现不典型,可累及额叶、脑干及脑深部结构,MRI检查是发现RPLS病变的最佳方法。     

可逆性后部白质脑病综合征1例报道

<正>可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)是一种少见疾病,是近年来逐渐被认识的一组临床-脑影像学综合征,主要表现为大脑后部以白质为主的可逆性病变,尤其当发生在产科等非神经科时,临床易误诊甚至导致不良后果。现将我院收治的1例可逆性后部白质脑病综合征病人报道如下。     

子痫前期相关性可逆性后部白质脑病综合征MRI影像学表现的研究进展

可逆性后部白质脑病综合征（RPLS）是一个由多种病因引起的临床影像学综合征，在产科中最常见的病因为子痫前期或子痫。子痫前期相关性RPLS是妊娠期高血压疾病的常见并发症之一，可威胁孕产妇和胎儿的生命安全。子痫前期相关性RPLS孕妇的影像学特征为大脑后部白质区血管源性水肿，MRI是诊断子痫前期相关性RPLS首选的影像学检查手段，常规的MRI检查序列包括T1加权像、T2加权像和T2液体衰减反转恢复序列。当发现脑部病变时，还需加扫弥散成像（DWI）序列，通过结合DWI和表观弥散系数图明确病变类型。必要时还可采取MRI血管成像、MRI静脉成像及磁敏感加权成像技术，排除其他颅脑血管疾病。此外，体素内非相干性运动、定量磁化率成像、MR波谱成像等MRI新兴技术也已用于子痫前期相关性RPLS脑功能及代谢情况的研究。这些MRI技术能够为子痫前期相关性RPLS的早期诊断和预后评估提供重要的影像学依据。     

Reversible posterior leukoencephalopathy associated with postpartum HELLP syndrome

We describe a case of a severe, immediate postpartum HELLP syndrome (hemolysis, elevated liver enzyme levels, low platelet count) with reversible posterior leukoencephalopathy (RPLS) characterized by generalized seizure, altered mental status, and visual loss in a 37-year-old primigravid woman. Magnetic resonance brain imaging showed diffuse subcortical edema, which resolved completely after 2 weeks along with complete symptom regression. The pathogenesis of RPLS is discussed and the importance of a prompt diagnosis is emphasized, as is the crucial role of rapid blood pressure reduction.     

Henoch–Schönlein purpura nephritis complicated by reversible posterior leukoencephalopathy syndrome

We report a young female patient with Henoch–Schönlein purpura (HSP) nephritis complicated by reversible posterior leukoencephalopathy syndrome (RPLS). The patient suddenly showed generalized seizures and cortical blindness with severe hypertension due to renal insufficiency approximately 1 year after cessation of corticosteroid treatment for HSP nephritis. Magnetic resonance imaging (MRI) demonstrated bilateral abnormal signals mainly in the cerebellum and white matter of the occipital lobe. Clinical symptoms quickly improved in conjunction with disappearance of abnormal signals on brain MRI after starting control of hypertension and continuous hemodiafiltration with steroid pulse therapy and plasmapheresis. RPLS may be caused by vasculitis and also by hemodynamic change due to severe hypertension in HSP, particularly in patients with nephropathy. In such cases intensive treatment should be performed as soon as possible to avoid neurological sequelae.     

Reversible posterior leukoencephalopathy secondary to indinavir-induced hypertensive crisis: a case report

Reversible posterior leukoencephalopathy syndrome (RPLS) is an uncommon entity related to multiple and different pathologies, the most common being hypertensive crisis. It is believed to be secondary to the breakdown on the blood-brain barrier. At the beginning, it is undistinguishable from other leukoencephalopathies. However, the disappearance of brain lesions after removal of the potential cause, establish the differential diagnosis with other leukoencephalopathies. We present the case of an HIV-infected patient with a RPLS related to a hypertensive crisis short after the initiation of indinavir-containing highly active antiretroviral therapy. Once blood pressure was controlled and indinavir replaced by nelfinavir, white matter lesions at magnetic resonance imaging disappeared. The clinical and radiologic evolution excludes other diagnosis as progressive multifocal leukoencephalopathy and points indinavir as a potential hypertension-inducing agent in HIV-infected predisposed subjects.     

Reversible posterior leukoencephalopathy syndrome after repeat intermediate-dose cytarabine chemotherapy in a patient with acute myeloid leukemia

A 56-year-old man was admitted to our hospital with leukocytosis, anemia, and thrombocytopenia. Acute monoblastic leukemia was diagnosed. Two subsequent courses of consolidation chemotherapy consisted of conventional doses of cytarabine and intermediate-dose cytarabine. Intermediate-dose cytarabine was infused intravenously every 12 hr for 6 days. On day 15 after the final infusion of cytarabine, the patient suffered headache, and on day 21, he experienced a decrease in sensation on the sole of his left foot. Magnetic resonance imaging (MRI) of the brain revealed widespread areas of white matter edema. Cerebrospinal fluid (CSF) examination revealed an increase in the number of cells to 31 mm(-3); the majority were lymphocytes. No infiltration of leukemia cells was seen. After 2 months, brain MRI findings were normal. The clinicoradiologic features of the case were consistent with reversible posterior leukoencephalopathy syndrome (RPLS). RPLS in the present case was unlikely to have been caused by direct neurotoxicity because (1) the doses of cytarabine (500 mg/m(2); total dose 9.2 g) were much smaller than those in reported cases and were repeatedly infused until RPLS developed; (2) the RPLS developed 21 days after the final infusion of cytarabine, a much longer period than previously reported; (3) the slight leukocytosis in the CSF observed on day 33 might also have been related to the cellular immune responses evoked by the infused cytarabine. These details suggest not only that direct cerebral neurotoxicity of cytarabine but also that some type of allergic response may have been involved in the development of RPLS.     

Clinical features of reversible posterior leukoencephalopathy syndrome in patients with systemic lupus erythematosus

To characterize reversible posterior leukoencephalopathy syndrome (RPLS) in systemic lupus erythematosus (SLE) in terms of treatments for resolution and its clinical course, we reviewed 28 cases of RPLS in SLE including our cases in view of the treatment. Of these, 15 cases improved with blood pressure control and 13 required immunosuppressive therapy for activity of SLE presenting neurological manifestations. Patients without immunosuppressants at onset of RPLS more frequently required immunosuppressive therapy to recover it than those precedingly using these agents [31% (4/13) versus 87% (13/15), p = 0.008, chi-square test]. Brain magnetic resonance imaging (MRI) is important for diagnosis of RPLS-SLE in the patient with SLE who develops neurological disturbance and rapidly increasing blood pressure. When 7-day therapy for hypertension and convulsion does not reverse the manifestations, immunosuppressive treatments would be recommended to reverse RPLS.     

Clinical features of reversible posterior leukoencephalopathy syndrome in patients with systemic lupus erythematosus

Abstract

To characterize reversible posterior leukoencephalopathy syndrome (RPLS) in systemic lupus erythematosus (SLE) in terms of treatments for resolution and its clinical course, we reviewed 28 cases of RPLS in SLE including our cases in view of the treatment. Of these, 15 cases improved with blood pressure control and 13 required immunosuppressive therapy for activity of SLE presenting neurological manifestations. Patients without immunosuppressants at onset of RPLS more frequently required immunosuppressive therapy to recover it than those precedingly using these agents [31% (4/13) versus 87% (13/15), p = 0.008, chi-square test]. Brain magnetic resonance imaging (MRI) is important for diagnosis of RPLS-SLE in the patient with SLE who develops neurological disturbance and rapidly increasing blood pressure. When 7-day therapy for hypertension and convulsion does not reverse the manifestations, immunosuppressive treatments would be recommended to reverse RPLS.     

Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis

OBJECTIVES: Reversible posterior leucoencephalopathy syndrome (RPLS) may develop in patients with renal insufficiency, hypertension, and immunosuppression, and is managed by prompt antihypertensive and anticonvulsant treatment. Four patients with renal insufficiency and fluid overload associated with Wegener's granulomatosis (one patient) and systemic lupus erythematosus (SLE) (three patients) are described, whose clinical picture and neuroimaging indicated RPLS. CASE REPORTS: All patients had headache, seizures, visual abnormalities, and transient motor deficit, and were hypertensive at the onset of the symptoms. Head computed tomography (CT) scan and magnetic resonance imaging showed predominantly posterior signal abnormalities, which were more conspicuous on T(2) weighted spin echo images than on CT scan. All patients had some form of cytotoxic treatment shortly before the syndrome developed, and dramatically responded to blood pressure control and anticonvulsant treatment. In two patients with SLE, dialysis was required for renal insufficiency. DISCUSSION: Follow up neuroimaging studies showed almost complete resolution of signal abnormalities, and suggested that RPLS was associated with cerebral oedema without concomitant infarction. The treatment of hypertension and neurotoxic condition such as uraemia appears of primary importance, while immunosuppressive treatment may cause further damage of the blood-brain barrier.     

系统性红斑狼疮合并可逆性脑白质后部综合征1例报道

目的 探讨系统性红斑狼疮（SLE）合并可逆性脑后部白质综合征（RPLS）的诱发因素、临床特点及诊治要点,以提高临床医生对该病的认识,降低误诊率。方法 对近期我科收治的1例SLE合并RPLS的临床资料进行了深入分析,并复习相关文献。结果 患者原发病诊断明确,入院后双手颤抖伴癫痫失神发作,予以大剂量激素联合他克莫司治疗及抗癫痫处理后效果不佳,停用他克莫司及积极控制血压等处理后,未再出现类似症状,头颅MRI提示病变范围较前明显缩小,综合考虑诊断为SLE合并RPLS。结论 SLE合并RPLS极易与神经精神狼疮、高血压脑病等疾病混淆,临床应提高对该病的认识和警惕,避免误诊误治。     

Hodgkin's disease-related central nervous system angiopathy presenting as reversible posterior leukoencephalopathy

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome characterized by headache, conscious disturbance, seizure, and cortical visual loss with neuroimaging finding of edema in the posterior regions of the brain, with a reversible course (1). The precise pathomechanism of RPLS is not understood. However, association with uncontrolled hypertension, renal failure, eclampsia, or immunosuppressive agents has been implicated (1). We describe herein a case of Hodgkin's disease (HD)-related central nervous system (CNS) angiitis with neuroimaging finding suggestive of RPLS. The pathophysiology of RPLS in cases with CNS angiitis is discussed.