Reversible Posterior Leucoencephalopathy Syndrome Research Articles

Reversible brain lesion following growth hormone replacement therapy in an adolescent

A 12.6-year-old girl presented with a 2-month history of headache, recurrent vomiting and 5 kg weight loss. She had been receiving recombinant human growth hormone (rhGH) replacement therapy at a...

Evaluation of fundus findings in preeclampsia [Preeklampside fundus bulgularinin degerlendirilmesi

In this study aimed to evaluate of the fundus examination findings in the patients with preeclampsia. Fundus findings of 45 patients hospitalized in Mersin University Hospital due to preeclampsia and consulted with blurred vision between June 2007 and February 2016 were evaluated retrospectively. The average age of patients was 28.6 ± 6.18 years and mean gestational age was 32.95 ± 4.22. Anterior segment examination was normal in all patients. The fundus findings of thirty five patients were assessed as normal, but pathological findings were detected in ten (%22.2) patients. Unilateral or bilateral serous retinal detachment was observed in six (%60) patients. Retinal hemorrhages and exudates were seen in four (%40) patients. In addition to the fundus findings reversible posterior leucoencephalopathy syndrome was detected in 5 patients. Six patients were delivered vaginally while thirty-nine patients with cesarean section. Serous retinal detachment and fundus findings regressed after delivery in patients. The preeclampsia syndrome is a multisystem disorder and it also can affect the visual pathways. Visual symptoms in preeclampsia include photopsia, visual field defects, blurred vision and, in severe cases, serious blindness. Blurred vision is the most common visual complaint. Hypertensive retinopathy, exudative retinal detachment and cortical blindness can be seen in patients. But in fundus examination pathological findings are not observed in all of the patients admitted with vision loss. Preeclampsia related retinal findings generally resolves after delivery and no additional treatment is required.

Neuropathies associated with oxaliplatin therapy

Neuropathies associated with oxaliplatin therapy

Reversible posterior leucoencephalopathy syndrome mimicking low-grade glioma: Differentiation by positron emission tomography

Reversible posterior leucoencephalopathy syndrome (RPLS) ypically presents with global encephalopathy, seizures, headache nd visual symptoms and is often triggered by a hypertensive mergency. Neuroimaging shows reversible vasogenic subcortial edema without infarction, preferentially of the posterior brain egions. In some patients, the RPLS lesions have a very asymmetrical nd even unilateral distribution [1].

Reversible posterior leucoencephalopathy syndrome in an elderly male on sunitinib therapy

Reversible posterior leucoencephalopathy syndrome (RPLS) has been reported following the use of anti-vascular endothelial growth factor (VEGF) agents such as bevacizumab, sorafinib and sunitinib. In this report we present a case of RPLS that occurred in an elderly male on sunitinib therapy. Other case reports of sunitinib-induced RPLS were reviewed and causality assessment was carried out using the World Health Organization-Uppsala Monitoring Centre criteria and the Naranjo algorithm. Only a few cases of sunitinib-induced RPLS had been reported previously and elevated blood pressure at presentation was common in most of the patients. Our case was clinically similar to the earlier reports and the adverse reaction had a 'probable' relationship with sunitinib intake. Physicians should monitor and manage elevated blood pressure in patients with sunitinib-induced RPLS.

Reversible Posterior Leucoencephalopathy Syndrome Following Duloxetine: A Case Report and Review of Literature

Reversible Posterior Leucoencephalopathy Syndrome (RPLS) is a disorder characterized by headaches, confusion, and/or decreased level of consciousness, seizures, and visual changes. A 58-year-old female was found unresponsive, with generalized rigidity and urinary incontinence for unknown duration. On presentation, she was febrile, tachycardic, tachypneic, and hypertensive. Neurological exam showed increased muscle tone and brisk deep tendon reflexes. Brain MRI revealed gyriform edema and white matter hypodensities in parieto-occipital regions consistent with RPLS. Discontinuation of duloxetine, one of her home medications, led to resolution of symptoms. We report this case to discuss the syndrome of RPLS and propose the role of duloxetine in causing hypertension and cerebral vasoconstriction leading to RPLS. To our knowledge, this is the first reported case of RPLS resulting from the use of duloxetine. doi:10.4021/jmc98w

Neuropsychiatric lupus: clinical challenges, brain-reactive autoantibodies and treatment strategies

Neurological manifestations in lupus can be due to active lupus disease affecting the brain or to other reasons. Reversible posterior leucoencephalopathy syndrome, primary lymphoma of the central nervous system, cerebral infections by bacteria (e.g. mycobacteria), viruses (e.g. JC virus), fungi (e.g. Cryptococcus) and parasites (e.g. Acanthamoeba), steroid-induced psychosis and reactive depression need to be excluded. Brain-reactive autoantibodies have been described as associating with neuropsychiatric lupus. The strongest associations described to date are with antiribosomal P protein and antiphospholipid antibodies. However these autoantibodies have not been shown to play significant roles in the pathogenesis. Treatment strategy for severe neuropsychiatric lupus include establishing definitive diagnosis, early identification and treatment of aggravating factors, appropriate symptomatic treatment, adequate immunosuppression, selective B-cell depletion and autologous haematopoietic stem cell transplant. Systematic reviews have shown that cyclophosphamide administration is superior to pulse methylprednisolone as a maintenance therapy. Mycophenolate mofetil has been shown to have modest effect and should only be considered if cyclophosphamide cannot be administered.

Preliminary safety data from a randomized trial of perioperative epirubicin, cisplatin plus capecitabine (ECX) with or without bevacizumab (B) in patients (pts) with gastric or oesophagogastric junction (OGJ) adenocarcinoma.

4019 Background: Perioperative chemotherapy is a standard of care for localised oesophagogastric cancer. B is a monoclonal antibody targeting vascular endothelial growth factor (VEGF), a potent regulator of angiogenesis. Tumour VEGF expression and high preoperative serum VEGF are independent predictors of poor prognosis for resected gastric cancer. ST03 is an ongoing randomised phase II/III trial of perioperative ECX ± B in pts with operable gastric or OGJ adenocarcinoma. Methods: Pts receive 3 preoperative and 3 postoperative ECX ± B 7.5 mg/kg, then 6 × maintenance B in the experimental arm only. The Independent Data Monitoring Committee undertook a preplanned review of preliminary safety data relating to preoperative chemotherapy and surgery for the first 104 pts. We present these data here to reassure clinicians and patients involved in the trial. Results: 43 ECX and 42 ECX + B pts have completed preoperative chemotherapy, 38 (88%) and 37 (88%) respectively received all 3 cycles. Of the 10 pts who received < 3 cycles, 7 (3 ECX, 4 ECX+B) stopped due to toxicity. B was administered in all preoperative cycles in the ECX + B arm. 7 ECX and 4 ECX + B patients experienced a total of 12 notable adverse events during preoperative chemotherapy: venous thromboembolic events (5 ECX, 1 ECX+B), gastric perforations (1 ECX, 1 ECX+B), haemorrhagic events (1 ECX, 1 ECX + B), hypertension (1 ECX + B) and wound complication from previous laparoscopy (1 ECX + B). Surgical data on the first 104 pts are immature but will be complete at the time of presentation. 51 (27 ECX, 24 ECX + B) pts have undergone gastrectomy. 6 pts (3 ECX, 3 ECX + B) had postoperative wound healing complications. 2 ECX + B pts had other notable events (reversible posterior leucoencephalopathy syndrome possibly due to B + microangiopathic haemolytic anaemia due to disease progression in the bone marrow). Conclusions: Preliminary safety data indicate acceptable and similar rates of complications in the 2 arms. 154 pts have been randomised to Jan. 8, 2010; safety and feasibility of ECX + B will be formally assessed when 200 pts have completed preoperative treatment. Author Disclosure Employment or Leadership Position Consultant or Advisory Role Stock Ownership Honoraria Research Funding Expert Testimony Other Remuneration Roche Roche Roche Roche

Chemotherapy‐induced reversible posterior leucoencephalopathy syndrome

Reversible posterior leucoencephalopathy syndrome is a neurological condition seen in various areas of acute medicine, including the administration of antineoplastic therapies used in haemato-oncology patients. It is a rare complication that has been increasingly recognized. It is characterized by altered mental status, visual disturbance, headache and seizures. Magnetic resonance imaging typically shows vasogenic oedema in the posterior regions of the brain. Although its name suggests reversibility, it may result in an irreversible brain injury without prompt treatment. Therefore, it is vital for treating clinicians to recognize this syndrome. We describe the case of a 55-year-old woman with advanced pancreatic adenocarcinoma, who developed clinical and radiological manifestations consistent with this syndrome as a complication of gemcitabine monotherapy.

Reversible posterior leucoencephalopathy syndrome associated with anticancer drugs

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. 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. 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 T(2)-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. 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 Cerebral Vasoconstriction Syndrome with Posterior Leucoencephalopathy after Oral Contraceptive Pills

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset recurrent 'thunderclap' headaches with reversible multifocal narrowing of the cerebral arteries, often associated with focal neurological deficits from ischaemic or haemorrhagic stroke. It has been associated with exposure to vasoconstrictive drugs, pregnancy, migraine, and a variety of other conditions. Whereas the pathophysiology of RCVS remains unclear, changes in the levels of female hormones are considered important because RCVS predominantly affects women and is frequently associated with pregnancy. We report a patient with angiographically confirmed RCVS whose MRI showed reversible brain oedema, suggesting an overlap between RCVS and the reversible posterior leucoencephalopathy syndrome. The only identified risk factor was oral contraceptive pills started 1 month prior to onset, supporting a role for female reproductive hormones in precipitating this overlap syndrome.

Atypical case of reversible posterior leucoencephalopathy syndrome associated with puerperal HELLP syndrome

Reversible posterior lekoencephalopathy syndrome (RPLS) is usually reversible. However, permanent cerebral damage may result if diagnosis is delayed. White matter edema in the posterior cerebral hemispheres is typical on neuroimaging. A 36-year-old primigravid woman underwent induction of labor due to mild pregnancy-induced hypertension. At 5 h after delivery, she developed eclampsia seizures complicated by hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. Magnetic resonance imaging showed high-density lesions in anterior regions without any abnormalities in posterior cerebral regions. Cases of postpartum RPLS without involvement of posterior brain regions after eclampsia complicated by HELLP syndrome are very rare. Patients with RPLS do not always show typical manifestations.

Neuropsychiatric lupus and reversible posterior leucoencephalopathy syndrome: a challenging clinical dilemma

Reversible posterior leucoencephalopathy syndrome (RPLS) has been increasingly recognized and reported in the literature. While the condition has been well described in patients with acute hypertension, pre-eclampsia, eclampsia, post-transplantation and chemotherapy, RPLS has been increasingly identified in patients with autoimmune diseases such as systemic lupus erythematosus (SLE). Though experience in the diagnosis and management of RPLS in patients with SLE is likely accumulating, few have systematically worked out the strategy to distinguish RPLS from neuropsychiatric SLE (NPSLE) and lupus-related complications of the central nervous system (CNS). Prompt recognition of, and differentiation between, these conditions is essential since their clinical presentations substantially overlap and yet their management strategy and subsequent outcomes can be entirely different. Indeed, inappropriate treatment such as augmentation of immunosuppression may be detrimental to patients with RPLS. A high index of suspicion of RPLS, prompt magnetic resonance imaging of the brain, including diffusion imaging, exclusion of CNS infection and metabolic derangement, a comprehensive medication review accompanied by timely and aggressive control of blood pressure and seizure are keys to successful management of RPLS. Such treatment strategy ensures a very high chance of total neurological recovery in lupus patients with RPLS.

Sickle cell disease complicated by post‐streptococcal glomerulonephritis, cerebral hemorrhage and reversible posterior leucoencephalopathy syndrome

A patient with homozygous hemoglobin SS disease presented with an intracerebral hemorrhage complicating reversible posterior leucoencephalopathy syndrome (RPLS), secondary to hypertension associated with acute post-streptococcal glomerulonephritis (APSGN). Distinguishing potentially reversible causes of central nervous system events from primary cerebral infarction or hemorrhage in patients with sickle cell disease is important because the management and prognosis of these complications is very different. Similarly, because of the difference in prognosis between APSGN and other forms of sickle cell nephropathy, it is also important to differentiate these conditions.

Transient Encephalopathy Complicating Poststreptococcal Glomerulonephritis in an Adult With Diagnostic Findings Consistent With Cerebral Vasculitis

We report the case of a young woman who had transient encephalopathy with nausea, cognitive impairment, 2 generalized seizures accompanied by visual impairment, and stenotic alterations of cerebral vessels lasting for weeks until complete resolution. These findings were associated with an elevated antideoxyribonuclease B level and biopsy-proven poststreptococcal glomerulonephritis. At the time of the encephalopathy, the patient had no electrolyte level disturbances, an only mildly elevated urea level, and moderate arterial hypertension and was on methylprednisolone therapy. For a couple of days, cranial magnetic resonance imaging showed multiple disseminated asymmetric hyperintensities on T(2)-weighted and fluid-attenuated inversion recovery, suggesting vasogenic edema. However, Doppler ultrasound examinations showed stenoses of extracerebral and multiple intracerebral arteries that persisted for several weeks, lasting considerably longer than the cerebral edema. This finding does not fit the context of hypertensive or steroid-induced encephalopathy, but is consistent with diagnosis of an accompanying vasculitis. Treatment with methylprednisolone for several weeks was associated with resolution of arterial stenoses and neurological symptoms, complete reversibility of Doppler sonographic findings, and significant improvement in renal function.

Reversible posterior leucoencephalopathy syndrome in a peripartum patient

We present the case of a multiparous parturient who developed hypertension associated with a severe headache in the immediate post-partum period. She subsequently suffered a generalised tonic clonic seizure on the fifth post-partum day. Following recovery of consciousness, she developed a left homonymous hemianopia. Apart from hypertension, headache and convulsion, she had no symptoms and no proteinuria or other biochemical or haematological changes associated with eclampsia. The magnetic resonance imaging findings were consistent with vasogenic oedema in the right posterior parieto-occipital white matter and these in turn are consistent with reversible posterior leucoencephalopathy syndrome. The differential diagnosis of convulsions in the post-partum period is discussed and the clinical and radiological features of reversible posterior leucoencephalopathy syndrome are described.

Paraparesis with complete recovery in antepartum eclampsia

Reversible posterior leucoencephalopathy syndrome (RPLES) is a well-known clinical entity that may be associated with an abrupt rise in blood pressure as in hypertensive encephalopathy and pre-eclampsia/eclampsia. The diagnosis of RPLES is suggested in retrospect by the reversal of neuroradiologic abnormalities and complete clinical recovery after treatment of the underlying cause. Studies have reported that RPLES invariably involves the posterior regions of the cerebral hemispheres especially the parieto-occipital lobes but that other areas may be involved as well. However a case of antepartum eclampsia with paraparesis attributed to leucoencephalopathy involving the frontoparietal regions of the cerebral hemispheres alone is being reported herein. (excerpt)

Leucoencefalopatía posterior reversible: descripción de un caso y revisión de la fisiopatología basada en hallazgos neurorradiológicos

Reversible posterior leucoencephalopathy is a clinico radiological syndrome first described in the last decade. The physiopathological mechanism governing it is not very well known. The currently accepted hypotheses are the generation of a vasogenic edema caused by failure of the mechanism that allows self regulation of the cerebral blood flow, and the production of a cytotoxic edema due to ischemia. Both experimental studies carried out in rats and the use of magnetic resonance imaging (MRI) to evaluate the diffusion of cerebral water support the first hypothesis. We report the case of a patient with reversible posterior leucoencephalopathy syndrome that was complicated by areas of cerebral infarction and we review the physiopathological mechanisms involved, basing our analysis on the findings obtained with MRI. A female patient was admitted to our hospital because of bilateral transient amaurosis, headaches, vomiting and arterial hypertension. MRI brain scans showed bilateral cortico subcortical lesions in the occipital parietal regions, frontal and right cerebellar hemisphere convexity, with hyperintense signal in T2 and diffusion weighted imaging. Some occipital parietal regions present a diminished signal on the apparent diffusion coefficient map. The patient was treated with intravenous antihypertensive drugs and full recovery was accomplished within 10 days. A month later, according to MRI scans, there were still hyperintense areas in T2 on both occipital lobes, which were compatible with cerebral infarcts. The mechanism that is most frequently involved in this entity is probably vasogenic edema. In our case there were, at the same time, areas of cytotoxic edema that progressed to cerebral infarcts. MRI with sequences that evaluate the diffusion of cerebral water is useful in distinguishing between the two mechanisms.

Hydrostatic brain edema: basic mechanisms and clinical aspect.

The term hydrostatic brain edema results from unfavorable hydrtostatic pressure gradiernts between blood vessels and brain tissue. Arterial hypertension combined with decompressive craniectomy produces extensive brain edema in the arterial boundary zone of the decompressive area. The increased hydrostatic pressure gradient enhances tissue damage and causes the biphasic opening of the blood-brain barrier (BBB). The hydrostatic pressure alone is capable of causing the initial BBB opening and this induced edema results in derangement of cerebral microcirculation and metabolism. With morphological opening and metabolic damage of the vascular wall, the second BBB opening is elicited by the amplified hydrostatic pressure gradient, which is similar to that in vasogenic edema. In the clinical aspect, the beneficial effect of decompressive craniectomy in the treatment of uncontrollable ICP and brain edema remains controversial. External decompression may have adverse effects on severe brain edema and swelling. In this regards, control of driving force for the formation of brain edema could be the treatment of choice as an initial step. In addition, recent reports provided by MR imaging indicated new information on the pathophysiological features of the patients with acute hypertension. In the patients with hypertension due to reversible posterior leucoencephalopathy (RPL) syndrome, MR images show reversible signal abnormalities in the bilateral occipital lobes, suggesting hydrostatic brain edema.

Hypertensive encephalopathy

The case report of a patient with hypertensive encephalopathy is presented here. The findings of reversible bilateral thalamic changes on T2-weighted MR imaging is an atypical manifestation of the reversible posterior leucoencephalopathy syndrome (RPLS), which is the neuroradiological correlate of hypertensive encephalopathy. The importance of prompt and accurate diagnosis of hypertensive encephalopathy / RPLS is stressed.