Reversible posterior leukoencephalopathy, severe hypertension, and cocaine abuse

Reversible posterior leukoencephalopathy syndrome is a brain disorder characterized by headache, nausea, vomiting, visual disturbance, depressed level of consciousness, convulsions and occasionally focal neurologic deficits. It is commonly associated with malignant hypertension, toxemia of pregnancy or the use of immunosuppressive agents. Early diagnosis and specific treatment is essential. We report a case of reversible posterior leukoencephalopathy in the context of a hypertensive crisis in an habitual cocaine sniffer. Reversible posterior leukoencephalopathy must be suspected in every patient with hypertensive crisis and compatible clinic manifestation. Neuroimaging studies show characteristic features which confirm the diagnosis.     

A case of reversible posterior leukoencephalopathy syndrome caused by transient hypercoagulable state induced by infection.

We report a normotensive case of reversible posterior leukoencephalopathy syndrome caused by transient hypercoagulable state. Hypertension is the main risk factor for reversible posterior leukoencephalopathy syndrome, which is believed to occur as a result of high blood pressure-related dysfunction of cerebrovascular endothelial cells, because it commonly appears in hypertensive emergency. However, in this completely normotensive case, the typical clinical findings of reversible posterior leukoencephalopathy syndrome were triggered by transient hypercoagulable state without any blood pressure variation. The case was successfully treated with anticoagulation therapy using heparin. Thus, this case indicates that reversible posterior leukoencephalopathy syndrome is induced by cerebrovascular endothelial dysfunction, which is induced not only by high blood pressure but also hemostatic dysfunction.     

Reversible posterior leukoencephalopathy in a patient with non-Hodgkin's lymphoma after treatment with CHOP

Reversible posterior leukoencephalopathy syndrome is a newly characterised and increasingly recognized clinico-radiologic syndrome. Underlying conditions that reportedly trigger this syndrome include hypertensive encephalopathy, eclampsia, renal failure, and immunosuppressive drug therapy with cyclosporine, tacrolimus and interferon alpha. We describe a 51-year-old woman with non-Hodgkin's lymphoma treated with conventional CHOP chemotherapy. Eight days after this treatment she developed severe headache, bilateral visual loss and focal seizures with secondary generalization. Neurologic examination showed confusion, cortical blindness, and left hemiparesis with hyperreflexia and sensory loss. A cranial T2-weighted magnetic resonance imaging revealed increased signal intensity in the occipital and frontal lobes in both hemispheres and right parietal lobe. A diagnosis of reversible posterior leukoencephalopathy was made. She presented a favourable outcome with conservative treatment with mannitol and phenytoin. A new cranial scanning showed nearly complete resolution of the abnormalities. To the best of our knowledge, this is the first case of reversible posterior leukoencephalopathy in a patient treated with standard-dose CHOP. In this patient, we confirm the theoretical pathophysiologic mechanisms suggested explaining how these drugs can cause the syndrome.     

Reversible posterior leukoencephalopathy in an HIV-infected patient with thrombotic thrombocytopenic purpura

We describe a 37-y-old male with advanced HIV disease who was diagnosed with thrombotic thrombocytopenic purpura after MRI of the brain revealed reversible posterior leukoencephalopathy. We discuss reversible posterior leukoencephalopathy as a diagnostic clue in HIV-infected patients with multi-organ system disease.     

Reversible posterior leukoencephalopathy syndrome in 2 HIV-infected patients receiving antiretroviral therapy.

We describe 2 human immunodeficiency virus-infected patients who developed hypertension and severe neurological abnormalities while receiving successful antiretroviral therapy. Neuroimaging findings were characteristic of reversible posterior leukoencephalopathy syndrome, a brain-capillary leak syndrome with hypertension and endothelial damage. We discuss the role of antiretroviral therapy-associated metabolic alterations in endothelial damage, hypertension, and reversible posterior leukoencephalopathy syndrome.     

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.     

可逆性后部白质脑病综合征的临床和影像学特征:9例回顾性病例系列研究

目的 探讨可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)的临床和影像学特征.方法 回顾性分析9例RPLS患者的临床和影像学资料.结果 继发于妊娠高血压综合征4例(44%),短肠综合征1例(11%),急性淋巴细胞白血病1例(11%)...     

Reversible posterior leukoencephalopathy syndrome probably caused by L-asparaginase

A 46-year-old male with refractory biphenotypic acute leukemia was treated with doxorubicin (days 1-3, 15-17), vincristine (days 1, 8, 15, 22), prednisolone (days 1-28), and L-asparaginase (L-ASP: days 15-28) as reinduction therapy. Physical examination revealed normotensive state and normal consciousness. On the 27th day, systemic seizures developed with mild hypertension (BP 151/98 mmHg). Computed tomography (CT) imaging of the brain showed areas of hypodensity in the bilateral white matter, and in the occipital and posterior parietal areas. Fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) showed some high intensity area involving the white matter, but also involving the cortex in the same area. Because the patient's condition progressed into unconsciousness and apnea from recurrent seizures, a respirator and anticonvulsants were needed. Four days later, the patient's general condition dramatically improved. There were no abnormal findings on MRI, and we diagnosed the cause of the seizures as reversible posterior leukoencephalopathy syndrome (RPLS). In adults, RPLS caused by chemotherapy is rare, especially L-ASP. Our patient did not have any previous history of convulsion up to the LAdVP, which brought on the seizures. It was considered that the RPLS might be caused by L-ASP, which had been given to this patient for the first time and was being given to him at the time of developing the seizures. RPLS is one of the causes of neurologic complications by L-ASP.     

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。     

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.