Reversible posterior leukoencephalopathy syndrome in a postpartum woman without eclampsia

We report a patient who developed reversible posterior leukoencephalopathy syndrome (RPLS) in puerperium without preeclampsia-eclampsia or chronic hypertension. The woman suddenly complained of visual loss and headache 10 days after delivery caused by edematous lesions mainly distributed in the bilateral occipital lobe. Apparent diffusion coefficient map was useful for distinction of this vasogenic edema from cytotoxic edema due to brain infarction. Under the diagnosis of RPLS, we successfully treated her disease using a trinitroglycerin as an antihypertensive, a hyperosmolar agent, methylprednisolone, and a free radical scavenger. Postpartum women may have the risk of development of RPLS even without preeclampsia-eclampsia. Vascular endothelial dysfunction may trigger RPLS, in addition to acute and modest increase in systemic pressure.     

Reversible posterior leukoencephalopathy syndrome in p-ANCA-associated vasculitis

The perinuclear antineutrophilic cytoplasmic antibody (p-ANCA) is closely associated with rapidly progressing glomerulonephritis, microscopic polyangiitis, and allergic granulomatous angiitis. While mononeuropathy due to vasculitis is a well-known neurological manifestation of these conditions, manifestations involving the central nervous system (CNS) have rarely been reported. Our patient presented the very characteristic CNS lesion of reversible posterior leukoencephalopathy syndrome (RPLS) which has often been associated with hypertension, eclampsia, cyclosporine neurotoxicity, and other diseases. The patient also developed the recently established disease entity, Takotsubo cardiomyopathy.     

Reversible posterior leukoencephalopathy syndrome in a patient with Takayasu arteritis

Reversible posterior leukoencephalopathy syndrome (RPLS) has been identified in several connective tissue diseases. However, there are no reports of RPLS associated with Takayasu arteritis (TA). We report the first case of TA associated with RPLS. A 23-year-old woman presented with sudden headache and vomiting, followed by generalized tonic–clonic seizures and mental changes two weeks after administration of oral prednisolone. MRI showed hyperintense signals on T2 and FLAIR images in the bilateral temporal–parietal–occipital lobes, left frontal lobe, and left cerebellar hemisphere. Three weeks after starting control of convulsions and blood pressure with plasmapheresis, high-dose methylprednisolone, and cyclophosphamide, the clinical manifestations and abnormal signals on MRI completely resolved. These reversible clinical and radiological changes are consistent with vasogenic edema in the central nervous system, indicating RPLS. Although high-dose methylprednisolone and cyclophosphamide are thought to cause RPLS, we think that it is justified to use these agents, at least in difficult cases, for making a clear-cut differentiation from CNS vasculitis, as long as blood pressure and fluid volume are well controlled. Moreover, we suggest that RPLS should be included in differential diagnosis of acute neurological changes in connective tissue diseases, including TA.     

Reversible posterior leukoencephalopathy syndrome: a misnomer reviewed

Reversible posterior leukoencephalopathy is a syndrome of headache, seizures and visual loss, often associated with an abrupt increase in blood pressure. Prompt diagnosis and therapy with antihypertensives, anticonvulsants, removal of any offending medication and treatment of associated disorders is essential since early treatment might prevent progression to irreversible brain damage. We present six illustrative cases presenting to Christchurch Hospital and review the condition. All were hypertensive, two were receiving immunosuppressant therapy after transplantation and one chemotherapy. Only three made a full recovery. The term reversible posterior leukoencephalopathy is a misnomer as the condition is not always reversible, is not necessarily confined to the posterior regions of the brain and can affect both white and grey matter. Magnetic resonance imaging findings of increased T2 and fluid attenuated inversion recovery signal predominantly involving the posterior regions of the cerebral hemispheres should alert the clinician to the possibility of this diagnosis.     

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.     

Reversible posterior leukoencephalopathy syndrome in a patient with Takayasu arteritis

Abstract

Reversible posterior leukoencephalopathy syndrome (RPLS) has been identified in several connective tissue diseases. However, there are no reports of RPLS associated with Takayasu arteritis (TA). We report the first case of TA associated with RPLS. A 23-year-old woman presented with sudden headache and vomiting, followed by generalized tonic–clonic seizures and mental changes two weeks after administration of oral prednisolone. MRI showed hyperintense signals on T2 and FLAIR images in the bilateral temporal–parietal–occipital lobes, left frontal lobe, and left cerebellar hemisphere. Three weeks after starting control of convulsions and blood pressure with plasmapheresis, high-dose methylprednisolone, and cyclophosphamide, the clinical manifestations and abnormal signals on MRI completely resolved. These reversible clinical and radiological changes are consistent with vasogenic edema in the central nervous system, indicating RPLS. Although high-dose methylprednisolone and cyclophosphamide are thought to cause RPLS, we think that it is justified to use these agents, at least in difficult cases, for making a clear-cut differentiation from CNS vasculitis, as long as blood pressure and fluid volume are well controlled. Moreover, we suggest that RPLS should be included in differential diagnosis of acute neurological changes in connective tissue diseases, including TA.     

Reversible posterior encephalopathy syndrome associated with bortezomib

Reversible posterior encephalopathy (RPES) is an uncommon neurological syndrome that is being increasingly reported in association with anti-neoplastic therapies. The first case of reversible posterior encephalopathy associated with the proteosome inhibitor bortezomib is described and the reported experience of the occurrence of RPES with other antineoplastic therapies reviewed. Dysregulation of cerebral vasomotor autoregulation is postulated as the underlying pathophysiology in this case of bortezomib associated RPES.     

Reversible posterior leukoencephalopathy syndrome complicating cytotoxic chemotherapy for hematologic malignancies

Reversible posterior leukoencephalopathy syndrome (RPLS) is an uncommon but distinctive clinicoradiological entity comprising of headache, seizures, visual disturbance, and altered mental function, in association with posterior cerebral white matter edema. With appropriate management, RPLS is reversible in the majority of cases. Previous reported associations of RPLS include hypertension, eclampsia, renal failure, and use of immunosuppressive drugs; reports in the adult hematology setting are rare. We report two cases of adults undergoing treatment for hematological malignancies who developed RPLS, and we emphasize the importance of early recognition and institution of appropriate management in reducing the risk of development of permanent neurological disability.     

Reversible posterior leucoencephalopathy syndrome associated with anticancer drugs

Background: Reversible posterior leucoencephalopathy syndrome (RPLS) is an underappreciated clinical‐radiologic syndrome characterized by reversible cortical dysfunction preferentially involving the occipital lobes in conjunction with imaging findings of reversible subcortical oedema. As RPLS is being increasingly identified within the oncology population in association with cytotoxic chemotherapy and targeted agents, a review of the published work in English was carried out. Methods: A MEDLINE search of the published work in English was conducted to identify cases of RPLS in patients more than 16 years of age who were treated with anticancer drugs for documented malignancy. Only cases with adequate documentation regarding demographic and treatment data, cerebral magnetic resonance imaging and outcome were selected. Results: We identified 24 patients with RPLS associated with a variety of anticancer drugs, most commonly complicating polychemotherapy and/or bevacizumab‐containing regimens. There was a female predominance: 18 females and 6 males (P= 0.023). Women were of premenopausal age and were younger than males: 49.3 ± 16.4 years versus 60.7 ± 6.4 years (P= 0.09). Most patients presented with acute headache (67%), seizures (63%), confusion (54%) or cortical blindness (46%) with mean systolic and diastolic blood pressure of 168 ± 15 and 98 ± 15 mm Hg, respectively. Findings on magnetic resonance imaging showed hyperintense lesions on T₂‐weighted images in all patients, which involved the occipital lobes in 75% of patients; all patients experienced clinical and radiologic resolution within days to weeks. No deaths were directly attributed to RPLS. Conclusions: Combination and single‐agent chemotherapy as well as novel anticancer drugs are associated with RPLS. We found RPLS to be overrepresented in premenopausal woman; the prevalence in this subgroup may be related to an anticancer drug–oestrogen interaction inducing altered cerebral vasoreactivity and endothelial dysfunction.     

Reversible posterior leukoencephalopathy syndrome and silent cerebral infarcts are associated with severe acute chest syndrome in children with sickle cell disease

Patients with severe acute chest syndrome (ACS) requiring endotracheal intubation and erythrocytopheresis are at increased risk for neurologic morbidity. This study examines patients with sickle cell disease who developed severe episodes of ACS, leading to endotracheal intubation, ventilatory support for respiratory failure, and erythrocytapheresis. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) studies, a neurologic examination by a pediatric neurologist, and cognitive testing were done in all patients. Five consecutive patients, aged 3 to 9 years, were identified with severe ACS. All patients developed neurologic complications resulting from ACS episodes, including seizures (n = 2), silent cerebral infarcts (n = 3), cerebral hemorrhage (n = 2), and reversible posterior leukoencephalopathy syndrome (n = 3). Children with severe ACS should have a magnetic resonance image of the brain, neurologic examination by a neurologist, and cognitive testing to detect the presence of neurologic morbidity.     

Reversible posterior leukoencephalopathy in patients with systemic lupus erythematosus

BACKGROUND: The development of central nervous system (CNS) symptoms in patients with preexisting systemic lupus erythematosus (SLE) evokes a wide differential diagnosis. Reversible posterior leukoencephalopathy (RPLE) is a rapidly evolving neurologic syndrome with characteristic clinical and radiographic features. Conditions commonly associated with RPLE include hypertensive encephalopathy, eclampsia, immunosuppressive drugs, and inflammatory disorders. OBJECTIVES: To describe our experience with RPLE in patients with concomitant SLE and review the literature. METHODS: The details of 5 novel cases and a MEDLINE review of the literature concerning the development of RPLE in association with SLE are presented. RESULTS: All cases included patients with SLE who developed the acute onset of headache, altered mental status, visual changes, and seizures. Neuroimaging demonstrated posterior white matter edema involving the parietal, temporal, and occipital lobes. Complete clinical and radiographic recovery occurred with prompt antihypertensive treatment and supportive care. Literature review identified 16 additional cases of RPLE occurring in patients with active SLE; the majority of these reports was similar in presentation and outcome to our experience. CONCLUSIONS: It is likely that the clinical manifestations and neuroimages in these lupus patients were the result of the RPLE syndrome. Fortunately, this cause of "secondary" CNS symptoms in patients with SLE is readily reversible when diagnosed early and treated with blood pressure control and supportive care.     

Reversible posterior leukoencephalopathy syndrome induced by bevacizumab plus chemotherapy in colorectal cancer

Reversible posterior leukoencephalopathy syndrome(RPLS)is a rare brain-capillary leak syndrome,characterized by clinical symptoms of headache,visual loss,seizures and altered mental functioning.This syndrome is usually reversible and is associated with hypertension,nephropathy,and use of immunosuppressive medication and cytotoxic agents.We describe two rare cases of RPLS occurring in colorectal cancer,both of which presented with coma,that we believe can be directly attributed to bevacizumab,a monoclonal antibody that inhibits the angiogenesis of tumours by specifically blocking vascular endothelial growth factor.We analysed the clinical features,risk factors and outcomes of RPLS in these two patients,and although no typical finding was identified on imaging examination,we found that inadequate blood pressure control was one of the risk factors leading to RPLS and that supportive treatment including intensive blood pressure control improved outcomes.Due to the increasing use of bevacizumab in colorectal cancer,clinicians should be aware of this potential complication.