Occipital Lobe Seizures as the Major Clinical Manifestation of Reversible Posterior Leukoencephalopathy Syndrome: Magnetic Resonance Imaging Findings

Summary: Purpose: Reversible posterior leukoencephalopathy syndrome (RPLS) is an increasingly recognized brain disorder most commonly associated with malignant hypertension, toxemia of pregnancy, or the use of immunosuppressive agents. When associated with acute hypertension, RPLS typically occurs concurrently with the fulminant clinical syndrome of hypertensive encephalopathy. We describe occipital lobe seizures, in the setting of only moderate elevations of blood pressure, as the major clinical manifestation of RPLS.
Methods: Two patients from the Dent Neurologic Institute are presented with clinical and magnetic resonance imaging(MRI) correlation.
Results: New onset secondarily generalized occipital seizures were noted, with MRI findings consistent with RPLS. Both of the patients had chronic renal failure and a moderate acute exacerbation of chronic hypertension. Other features of hypertensive encephalopathy were lacking, such as headache, nausea, papilledema, and an altered sensorium. Magnetic resonance imaging (MRI) showed edematous lesions primarily involving the posterior supratentorial white matter and corticomedullary junction, consistent with RPLS. With lowered blood pressure, the MRI lesions resolved and the patients became seizure-free without requiring chronic anticonvulsant therapy.
Conclusions: Occipital seizures may represent the only major neurologic manifestation of RPLS due to acute hypertension, especially in patients with renal failure. Other evidence of hypertensive encephalopathy, such as cerebral signs and symptoms, need not be present. Blood pressure elevations may be only moderate. Early recognition of this readily treatable cause of occipital seizures may obviate the need for extensive, invasive investigations. Despite the impressive lesions on MRI, prompt treatment of this disorder carries a favorable prognosis.     

Reversible posterior leukoencephalopathy syndrome: a case presentation

Reversible posterior leukoencephalopathy syndrome (RPLS) is encountered in many subspecialty areas of medical practice but is seen relatively infrequently by the neurodiagnostic technologist. There are several different names for this condition including: reversible posterior leukoencephalopathy, reversible posterior leukoencephalopathy syndrome, posterior reversible encephalopathy syndrome, and hypertensive encephalopathy. A brief review of RPLS and the case presentation of a woman who developed eclampsia, temporary blindness, and recurrent seizures captured by EEG/video monitoring are presented in this paper EEG samples and brain magnetic resonance imaging (MRI) changes related to RPLS are highlighted.     

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 a patient with thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterised by fever, microangiopathic haemolytic anaemia, renal insufficiency, and thrombocytopenia. Neurological involvement, a prominent component of TTP, is characterised by a variety of brain lesions which include reversible cerebral oedema or magnetic resonance imaging (MRI) features of reversible posterior leukoencephalopathy syndrome (RPLS). TTP is frequently associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13.     

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.     

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.     

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.     

Two Cases of Hypertensive Encephalopathy Involving the Brainstem

Hypertensive encephalopathy is a medical emergency whose clinical manifestations are usually associated with bilateral parieto-occipital lesions. Predominant brainstem edema without accompanying occipital lesions is rare in hypertensive encephalopathy and usually occurs in patients with secondary hypertension. We describe the clinical and radiological features of two patients with reversible hypertensive brainstem encephalopathy. Both patients had chronic renal failure, but the extensive neuroimaging abnormalities revealed few clinical features of brainstem involvement. The clinical findings and neuroimaging abnormalities resolved once the hypertension was treated.     

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。     

Postpartum angiopathy with reversible posterior leukoencephalopathy

BACKGROUND: Postpartum angiopathy (PPA) is a cerebral vasoconstriction syndrome of uncertain cause that affects large and medium-sized cerebral arteries. Postpartum angiopathy is frequently complicated by ischemic stroke. The reversible posterior leukoencephalopathy syndrome (RPLS) is a distinct clinical-radiological entity characterized by transient vasogenic edema on brain imaging. The pathophysiological features of RPLS are related to small-vessel dysfunction and breakdown of the blood-brain barrier. OBJECTIVES: To report the coexistence of PPA and RPLS in 4 patients and to discuss possible interrelationships between these 2 entities. DESIGN: Four case reports and a review of the literature. RESULTS: Four women developed a clinical-radiological syndrome overlapping PPA and eclampsia shortly after an uncomplicated pregnancy. All had acute severe ("thunderclap") headaches and hypertension. Three developed seizures. All patients had reversible angiographic narrowing of large and medium-sized cerebral arteries. Serial magnetic resonance imaging showed transient nonischemic brain lesions, resembling the lesions described in patients with RPLS. The results of extensive tests for cerebral vasculitis were negative. CONCLUSION: These cases, and the literature, suggest an interrelationship between RPLS and cerebral vasoconstriction syndromes such as PPA.     

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.     

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。     

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。     

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.     

可逆性后部白质脑病综合征临床及影像学特点分析（附六例报道）

目的 探讨可逆性后部白质脑病综合征的临床及影像学特点。 方法 收集无锡市人民医院神经内科自2007年12月至2010年8月诊治的6例可逆性后部白质脑病综合征患者的临床资料,回顾性分析其发病原因、临床症状、影像学特点、治疗及预后。 结果 6例患者中白血病1例,肾病综合征1例,肾移植1例,嗜铬细胞瘤1例,原发性高血压2例。患者临床表现多样,主要为痫性发作、头晕头痛、恶心呕吐、意识障碍、精神行为异常、视觉障碍等。头颅CT检查示4例脑白质低密度病灶,2例正常。头颅MRI检查主要表现为大脑半球后部白质病变,TIWI呈低或等信号,T2WI呈高信号,液体衰减反转恢复序列(FLAIR)呈高信号,弥散加权成像(DWI)呈低信号或等信号,表观弥散系数图(ADC)呈高信号。5例经对因及对症治疗临床症状迅速改善,影像学表现减轻或恢复正常;1例肾移植患者因拒绝停用环孢霉素A及骁悉而最终死亡。 结论 可逆性后部白质脑病综合征是一组依靠病史、临床特征及影像学特点而诊断的疾病,经积极治疗大多预后较好,但也有小部分患者由于种种原因而预后不良。     

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。     

Reversible brainstem edema due to hypertensive encephalopathy in an 8-year-old girl

The authors report an 8-year-old girl with refractory status epilepticus due to hypertensive encephalopathy, secondary to end-stage renal disease. Brain magnetic resonance imaging (MRI) in the acute phase showed striking hyperintensities in the brain stem and medial thalamus along with subtle cortical lesions. After successful control of hypertensive crisis and status epilepticus, the patient recovered to her baseline. Near total resolution of the lesions was noted on follow-up imaging performed 9 days later. Predominant brainstem involvement as a feature of posterior reversible encephalopathy syndrome due to hypertensive crisis is extremely rare in children and has not been well documented.     

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。     

Interictal cortical reorganization in episodic migraine without aura: an event-related fMRI study during parametric trigeminal nociceptive stimulation

Reversible posterior leukoencephalopathy syndrome (RPLS) is theoretically associated with hypertensive encephalopathy because the most patients demonstrate abrupt increasing of blood pressure (BP). A 59-year-old woman, who had undergone cholecystectomy 4 days before, complained of a headache and rapidly progressing visual disturbance. Her BP was postoperatively controlled at around 150/80, but her BP was 89/46 when she noticed her symptoms. Magnetic resonance imaging showed vasogenic edema in bilateral occipital and right parietal lobes, and intracranial magnetic resonance angiography revealed bilateral diffuse peripheral vasoconstriction. After discontinuing ropivacaine administration via epidural catheter, her BP rose to 114/62 and her symptoms completely disappeared within a few days. Except for hypotension, the clinical course and the radiological evidences in our case were consistent with RPLE. This case supports another hypothesis of RPLS mechanism that arterial endothelial injury by toxic drug effect results in transudation of fluid from blood vessels causing vasogenic brain edema.