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Posterior Reversible Encephalopathy Syndrome: Associated Clinical and Radiologic Findings

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To identify and define clinical associations and radiologic findings of posterior reversible encephalopathy syndrome (PRES). Patients prospectively diagnosed as having PRES from October 1, 2005, through April 30, 2009, were pooled with retrospectively identified patients admitted from August 1, 1999, through September 30, 2005. We performed a detailed review of clinical information, including demographics, presenting symptoms, medical history, and risk factors. All patients underwent computed tomography of the brain or magnetic resonance imaging. Findings on magnetic resonance imaging were analyzed independently by 2 neuroradiologists. We identified 120 cases of PRES in 113 patients (mean age, 48 years). Mean peak systolic blood pressure was 199 mm Hg (minimum-maximum, 160-268 mm Hg), and mean peak diastolic blood pressure was 109 mm Hg (minimum-maximum, 60-144 mm Hg). Etiologies of PRES included hypertension (n=69 [61%]), cytotoxic medications (n=21 [19%]), sepsis (n=8 [7%]), preeclampsia or eclampsia (n=7 [6%]), and multiple organ dysfunction (n=1 [1%]). Autoimmune disease was present in 51 patients (45%). Clinical presentations included seizures (n=84 [74%]), encephalopathy (n=32 [28%]), headache (n=29 [26%]), and visual disturbances (n=23 [20%]). In the 115 cases (109 patients) for which magnetic resonance imaging findings were available, the parieto-occipital regions were the most commonly involved (n=108 [94%]), followed by the frontal lobe (n=88 [77%]), temporal lobe (n=74 [64%]), and cerebellum (n=61 [53%]). Cerebellar involvement was significantly more frequent in patients with a history of autoimmunity (P=.008), and patients with sepsis were more likely to have cortical involvement (P<.001). A substantial proportion of patients with PRES have underlying autoimmune conditions that may support endothelial dysfunction as a pathophysiologic mechanism. On brain imaging, the location and severity of vasogenic edema were mostly similar for the different clinical subgroups.

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... Additional risk factors in women include pregnancy, use of oral contraceptives, and hormonal therapy [1]. Stroke in pregnancy is relatively rare; however, certain risk factors may be associated with stroke in females during pregnancy these include age more than 35 years, black ethnicity, hypertension, heart disease, smoking, diabetes, lupus, sickle cell disease, migraine headaches, alcohol and substance abuse, caesarean delivery, fluid and electrolyte disorders, thrombophilia, multiple gestations, greater parity, and postpartum infection [1,2]. Furthermore, specific stroke syndromes in pregnancy include pre-eclampsia and eclampsia, subarachnoid hemorrhage secondary to aneurysmal rupture, cerebral venous thrombosis, paradoxical embolism, peripartum cardiomyopathy and postpartum cerebral angiopathy (PCA) (as the present case) [1]. ...
... On the other hand, PRES presents with T2 and FLAIR hyperintensities. The significant implication here is that the pathology of PCA involves cerebral infarction, while PRES involves cerebral edema brought about by cerebral endothelial dysfunction and leakage [2]. Clinical characteristics greatly overlap as well as both conditions may present with alterations in sensorium, seizures, headache, and focal neurologic deficits. ...
... Arterial abnormalities are better visualized with CT angiography and conventional invasive catheter angiography. The MRI and MRA features may normalize with time, although extensive infarction may develop [1][2][3][4]. PCA has overlapping clinical and angiographic features with cerebral vasculitis. Cerebrospinal fluid analysis may be helpful during diagnostic uncertainty, and this is often unremarkable in PCA. ...
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This is a case of a 32-year-old primigravid who developed sudden severe headache on the 7th day postpartum associated with focal neurologic deficits and altered sensorium. She had a GCS score of 6, anisocoric pupils and an NIHSS score of 31. Cranial MRI with MRA showed multifocal hyperacute to acute infarcts on the left occipital lobe, left thalamus, and midbrain which was more prominent on the right. Due to clinical deterioration, a repeat Cranial MRI with MRA was done and showed progression of infarcts involving both thalami and right pons with interval appearance of contour irregularities in the proximal anterior cerebral, posterior cerebral, basilar and internal carotid arteries. Serial transcranial Doppler showed significant distal right middle cerebral artery vasospasm. She was managed as a case of reversible cerebral vasoconstriction syndrome, associated with postpartum cerebral angiopathy. Intravenous pulse methylprednisolone was started subsequently IVIG was initiated. Intravenous immunoglobulin was given for 5 days. The patient gradually improved, underwent rehabilitation therapy, and was discharged stable after 6 weeks.
... In radiological imaging, abnormalities are seen in the posterior region of both cerebral hemispheres. 1 Though the pathophysiology of PRES is not clearly understood, but vasogenic edema secondary to disruption of blood-brain barrier is characteristic of it. There is no specific age group for this condition, but most of the cases occur in the third to fifth decade of life, with predominance in females. ...
... This finding is similar to a study by Fugate et al. that showed involvement of 65% of females. 1 In the present study, the mean age of onset is 38.6 years, which is comparable with other studies. 4 The most common clinical presentation is seizure (66%). ...
... These findings are comparable with other studies in literature. 1 In a study by Pedraza et al., PRES was most commonly reported with hypertension, preeclampsia and eclampsia, and HELLP syndrome. 12 In the present series, the most common etiologies are underlying kidney disease and eclampsia and postpartum sepsis. ...
Article
Background: Posterior reversible encephalopathy syndrome (PRES) is a reversible condition. The Main pathological feature is vasogenic cerebral edema with predominant involvement of posterior part of the cerebrum. Clinical symptoms range from headache, seizure, and vision loss. We evaluated the clinicoradiological features of patients with PRES and their clinical outcome. Materials and methods: A retrospective study with 30 cases from January 2014 to May 2017. Results: Of thirty patients, 18 were females and twelve patients were male. The Mean age of the patients was 38.6 years. The most common presentation was seizure (66.6%) followed by altered mental status (53.3%) and headache (40%). The Main comorbid illnesses in our study were renal disease (36.7%), hypertension (23.3%), eclampsia, and postpartum sepsis (26.7%). The Most common site was the occipito-parietal region in the magnetic resonance imaging brain (66%). Atypical presentation involved the temporal lobe (16%), basal ganglia (6%), and microhemorrhage (6%). The Outcome was good with 20% mortalities. Conclusion: PRES is a reversible condition and has a good outcome in most patients. In our study, seizure was the most common presentation and renal disease is the most common comorbid illness. How to cite this article: Goyal G, Jeswani J. Study of Clinicoradiological Profile in Posterior Reversible Encephalopathy Syndrome: An Experience from North India. Indian J Crit Care Med 2022;26(4):501-505.
... in 1996 [2], with symptoms of encephalopathy (92%) [3,4], seizures (74%) [4], hypertension (70%) [3][4][5], headache (26%) [3], and visual changes (20%) [3]. Classic neuroimaging findings involve cortical and subcortical posterior cerebral white matter edema best identified on T2 fluid-attenuated inversion recovery (FLAIR) imaging [2,[5][6][7]. ...
... in 1996 [2], with symptoms of encephalopathy (92%) [3,4], seizures (74%) [4], hypertension (70%) [3][4][5], headache (26%) [3], and visual changes (20%) [3]. Classic neuroimaging findings involve cortical and subcortical posterior cerebral white matter edema best identified on T2 fluid-attenuated inversion recovery (FLAIR) imaging [2,[5][6][7]. While incidence is not well-reported, it is likely under-diagnosed [4]. ...
... While incidence is not well-reported, it is likely under-diagnosed [4]. Risk factors include female gender, middle-age, autoimmune disease, immunosuppressive therapy, history of hypertension, renal disease, and eclampsia [5,6]. ...
Article
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Background Posterior reversible encephalopathy syndrome (PRES) represents a transient change in mental status with associated vasogenic edema of cortical and subcortical brain structures. It is often attributed to multifactorial etiology including hypertension and altered hemodynamics and disruption of vessel integrity. Patients with autoimmune disease and certain immune modulator therapies are at greater risk. Case presentation A 54-year-old female with past medical history of well-controlled multiple sclerosis on interferon-beta since 2013, presented with witnessed tonic colonic seizure. She also was noted to demonstrate left gaze deviation and left-sided hemiparesis. MRI fluid-attenuated inversion recovery sequence showed hyperintensity of the subcortical U fibers, concentrated in the occipital, parietal lobes and frontal lobes. Systolic blood pressure was 160 mmHg on arrival. The patient was started on seizure prophylxis and Interferon beta was discontinued. The patient’s mentation, seizures and hemiapresis significantly improved in next 72 h with tight blood pressure control, and had notble improvement on MRI imaging and inflammatory markers. Lumbar puncture CSF results were devoid of infectious and autoimmune pathology. Conclusions A middle-aged female with multiple sclerosis who was on chronic IFN-beta presented to the emergency room with a witnessed tonic-clonic seizure, with MRI T2 FLAIR imaging consistent with PRES. She had notable clinical improvement with decreased edema on imaging and improved inflammatory markers 72 h after cessation of IFN-beta therapy.
... Bilateral subcortical white matter vasogenic edema affecting parieto-occipital regions, defined as T2 fluid attenuation inversion recovery hypersensitivity, typically presents on magnetic resonance imaging (MRI) with PRES 2 . However, there may also be atypical occurrences with signals in the brainstem, basal ganglia, cerebellum, or temporal lobes or alternatively a lack of edema 3,4 . The pathophysiology of PRES is theorized to occur either by a rapid increase in arterial blood pressure causing cerebral hyperperfusion or endothelial dysfunction caused by circulating endogenous or exogenous toxins, with both causing vasogenic edema 5 . ...
... Significant neck pain and headaches One-day postsurgery chest anteroposterior x-ray indicating prominent bilateral atelectasis. 4,2022 made physical therapy untenable for the patient, with conservative treatment consisting of 2 prior C5-C6 epidural steroid injections. While offering relief for pain, there were concerns for iatrogenic exacerbation of the patient's osteoporosis, and systemic effects from repeat injections as well as demonstrated limited benefit for myelopathy 8,9 . ...
... This is postulated to create an unintended Valsalva maneuver after extubation with the laryngospasm, causing a retropharyngeal bleed that likely originated from the exposed bony surfaces and epidural veins. Timely recognition of PRES with treatment consisting of stepwise blood pressure control is crucial because the extent of vasogenic edema and hemorrhage has been associated with discharge disposition 4,6,17 . Despite the difficult circumstances that occurred after her surgeries and the PRES episode, the patient recovered without incident after the administration of nicardipine, with optimal outcome at follow-up. ...
Article
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Case: A 71-year-old woman with a 2-year history of spondylosis and radiculopathy presented with progressively worsening cervical spine pain and neurological dysfunction for 2 years. The patient developed posterior reversible encephalopathy syndrome (PRES) after postoperative hematoma in the retroesophageal and retropharyngeal areas. This occurred status post anterior cervical diskectomy and fusion (ACDF) of C4-C6. One year postoperatively, the patient recovered with full ambulation, neurological improvement, and resolution of all PRES signs and symptoms. Conclusion: We present a case of a previously undescribed outcome of PRES as a complication of ACDF likely related to postoperative hypertension and fragile blood vessels.
... Posterior reversible encephalopathy syndrome (PRES) is a syndrome characterized by reversible subcortical vasogenic edema in the brain that manifests in patients with acute neurological symptoms (i.e., seizures, encephalopathy, headache, and visual disturbances) [1][2][3][4][5][6]. It has been reported to occur in the setting of various systemic illnesses, such as renal failure, dramatic blood pressure fluctuations, cytotoxic drugs, autoimmune disorders, and preeclampsia or eclampsia [2,4,6]. ...
... Posterior reversible encephalopathy syndrome (PRES) is a syndrome characterized by reversible subcortical vasogenic edema in the brain that manifests in patients with acute neurological symptoms (i.e., seizures, encephalopathy, headache, and visual disturbances) [1][2][3][4][5][6]. It has been reported to occur in the setting of various systemic illnesses, such as renal failure, dramatic blood pressure fluctuations, cytotoxic drugs, autoimmune disorders, and preeclampsia or eclampsia [2,4,6]. Pediatric PRES may seldom occur and often arises in the setting of chemotherapy initiation for acute lymphocytic leukemia (ALL), which results in endothelial cytotoxic injury [4]. ...
... Although the pathogenesis is under debate, the leading theory purports that PRES is due to disruption in the autoregulation of cerebral perfusion, which subsequently results in the breakdown of the blood-brain barrier [1][2][3][4][5][6][7][8][9][10]. This allows the extravasation of plasma and macromolecules into the brain interstitial space [4,6]. ...
Article
Posterior reversible encephalopathy syndrome (PRES) is a disorder that most commonly affects adults, and is characterized by neurologic symptoms such as encephalopathy, seizures, headaches, and visual disturbances. It usually occurs in the context of other systemic disturbances that result in hypertensive crises, such as renal failure, cytotoxic drugs, and autoimmune conditions. In children, it rarely manifests following chemotherapy induction or hematopoietic stem cell transplantation. No cases have been reported in the English literature connecting renal dysfunction and hypertensive emergency secondary to post-streptococcal glomerulonephritis (PSGN) with PRES. We present a case of an eight-year-old boy, who developed a constellation of symptoms suggestive of PSGN and later developed PRES. PRES is often confirmed upon suspicion through brain MRI showing subcortical edema of various brain regions including occipital, temporal, or parietal cortices. Our patient demonstrated subcortical edema of the bilateral occipital lobes and right cerebellar hemisphere, with positive antistreptolysin O (ASO) titers demonstrating PSGN as the likely etiology for his hypertensive emergency. Management included antihypertensive and anticonvulsant treatment, which allowed the resolution of the offending hypertensive emergency that resulted in PRES. Our case adds to the growing body of literature on PRES and describes a new etiology of pediatric PRES secondary to PSGN.
... In the paediatric population, the incidence of cerebellar and brainstem involvement has been documented as 33-58% [4,21,22] but is considered an atypical imaging finding [9]. In our study, 12 (19%) children had cerebellar involvement. ...
... Frontal lobe involvement, which is also considered an atypical imaging finding, was significant in our patient population (40%), exceeding the number of cases that showed parietal and occipital lobe involvement. Results from previous studies have shown an even higher frontal lobe involvement of 54-81% in children with PRES [15,16,21]. ...
... Further, features that are considered atypical in the adult population may be typical in paediatric PRES and should therefore be part of the radiologic criteria. Another hypothesis to explain the pathogenesis of PRES is the direct cytotoxic effects of chemotherapeutic agents that result in endothelial damage, release of vasoactive agents, necrosis and damage to brain tissue [4,21,22]. We observed an increased incidence of PRES in cancer patients on chemotherapy (14 of 15 patients) at the time of clinical presentation. ...
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Background Posterior reversible encephalopathy syndrome (PRES) is relatively uncommon in paediatric patients; however, its pathophysiology remains obscure. Objective The aims of this study were to find an association or correlation between (1) blood pressures and (2) imaging severity and the presence of atypical imaging features in children with PRES. Materials and methods We performed a retrospective cross-sectional evaluation in children diagnosed with PRES. We reviewed radiologic findings along with each patient’s clinical profile and outcome. We categorised imaging severity into mild, moderate and severe, and assessed the MR imaging pattern, enhancement and diffusion restriction for each child. We assessed both associations and correlations between variables using the chi-square test, Cramer V and Kendall tau b. Results A total of 63 children met the inclusion criteria (31 boys; mean age 9.7 years). A total of 42 children (67%) had an elevated blood pressure. Imaging showed parieto-occipital lobe involvement pattern in 24 (38%) children, frontal lobe pattern in 25 (40%) and cerebellar involvement in 12 (19%). Three (5%) had haemorrhage, 15 (24%) had contrast enhancement and 19 (30%) had positive diffusion restriction (cytotoxic oedema). We found no statistically significant association between imaging severity and blood pressures (P=0.11), nor any association between blood pressure and atypical imaging findings such as diffusion restriction (P=0.1), enhancement (P=0.11) or haemorrhage (P=0.33). Conclusion According to our results, there is no statistically significant association or correlation between blood pressure and either imaging severity or atypical imaging features in children with PRES. Further prospective studies are warranted.
... Extremely elevated blood pressure will subsequently lead to failure of cerebral blood vessels autoregulation, increased cerebral perfusion pressure (CPP), and endothelial dysfunction. This endothelial injury and increased capillary permeability will lead to brain vasogenic edema resulting in the classical PRES symptoms and radiological findings [3]. ...
... The parietal and occipital lobes are the typical distribution. The frontal, inferior temporal-occipital junction and the cerebellum follow them in involvement frequency [3,17]. ...
... This syndrome is often associated with acute hypertension, which is commonly caused by acute kidney injury or eclampsia. 5,6 Although the exact pathophysiological mechanism of PRES remains unclear in most patients, this condition is accompa-nied by impaired renal function in the form of either chronic or acute kidney injury. Fugate et al. 6 reported that 38% of patients with PRES exhibit chronic kidney disease and that more than half exhibit chronic hypertension. ...
... 5,6 Although the exact pathophysiological mechanism of PRES remains unclear in most patients, this condition is accompa-nied by impaired renal function in the form of either chronic or acute kidney injury. Fugate et al. 6 reported that 38% of patients with PRES exhibit chronic kidney disease and that more than half exhibit chronic hypertension. Studies have demonstrated that acute pancreatitis may cause PRES. ...
... 3 It is mostly associated with episodes of hypertension before the symptoms present. 2 The exact pathophysiological mechanism of PRES is still unclear but the few proposed mechanisms are: (i) Severe hypertension overshoots the limits of autoregulation which results in vasogenic cerebral edema, 4 (ii) Cerebral vasoconstriction causing subsequent infarcts in the brain, (iii) Endothelial damage with blood-brain barrier disruption further leading to fluid and protein transudation in the brain. 5 PRES has also been associated with systemic inflammatory conditions like sepsis, transplantation, autoimmune states, and eclampsia. 6 Patients with chronic kidney disease or acute kidney injury are at higher risk for PRES as the incidence of hypertension is higher in these patients. ...
... 7 As COVID-19 attaches to angiotensinconverting enzyme (ACE) 2 receptors in lung and brain parenchyma, it probably induces changes in the renin-angiotensin aldosterone system (RAAS) and causes blood pressure fluctuations and disrupts the blood-brain barrier. 5,8 This could act as a trigger for PRES in COVID patients. ...
Article
Background: Coronavirus disease-2019 (COVID-19) infection-related neurological events are not uncommon but presenting as posterior reversible encephalopathy syndrome (PRES) without hypertension is a very rare presentation and requires a high index of suspicion. Case summary: We report a case of a middle-aged female who presented with severe COVID-19 disease with no neurological symptoms. She complained of diminished vision on day 7 of the illness and underwent an MRI brain to rule out an ischemic stroke but the findings were suggestive of PRES. She had no episode of hypertension during the hospital stay. Probably severe COVID-related inflammation was the reason for such a presentation. Conservative management resolved the issue and her symptoms weaned off. Conclusion: Severe COVID disease can lead to PRES-like symptoms and requires neuroimaging to validate it. Conservative management is the best treatment for such patients. How to cite this article: Sharma D, Tomar DS, Gupta S. Non-hypertension-associated Posterior Reversible Encephalopathy Syndrome in COVID-19. Indian J Crit Care Med 2022;26(5):641-642.
... PRES is an entity with a high mortality rate and risk of permanent neurological deficit. Death may result from progressive cerebral edema, intracerebral hemorrhage, or a complication of the underlying condition [2]. Antihypertensive drugs are the mainstay of PRES treatment. ...
... Antihypertensive drugs are the mainstay of PRES treatment. Besides, stopping or lowering the dose of the offending agent, anti-seizure medications, and treatment of other acute medical conditions (e.g., vasculitis, sepsis, renal failure) may also hasten patient's recovery [1][2][3]. ...
Article
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A 21-year-old woman with unremarkable past health presented with an intense headache and visual disturbance, followed by a secondary generalized tonic-clonic focal seizure. She had a history of arthritis, livedo reticularis, myalgia, elevated erythrocyte sedimentation rate, and nonspecific constitutional manifestations (fever and weight loss). Examination revealed new-onset severe hypertension. Complete immunological studies were negative. Electroencephalogram showed abnormalities compatible with secondarily generalized tonic–clonic focal occipital seizures. Brain magnetic resonance imaging (MRI) revealed high signal intensity on T2 in occipital lobes, cerebellum, and brainstem and renal imaging multiple hypoenhancing parenchymal images (renal infarction). Follow-up brain MRI at 3 months showed marked improvement. A diagnosis of polyarteritis nodosa with posterior reversible encephalopathy syndrome was made.
... Risk factors for PRES include hypertension, eclampsia, renal disease, organ transplantation, certain immunomodulatory therapies, autoimmune conditions, some chemotherapeutic medications, and sepsis (Bartynski, 2008;Fugate et al., 2010). Although PRES has been well described, the aetiology remains unclear. ...
... The onset of PRES has historically been considered to frequently be "multifactorial" (Hinchey et al, 1996, p. 499) with a range of clinical situations such as hypertension, eclampsia, renal disease, organ transplantation, certain immunomodulatory therapies, autoimmune conditions and sepsis (Fugate et al., 2010) implicated via mechanisms of disrupted cerebral autoregulation and/or damage to the endothelium (Fischer & Schmutzhard, 2017). Iftikhar et al (2021) conclude that it is likely that COVID-19 adds an additional risk factor to those who are already predisposed to develop PRES. ...
Preprint
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Objective: We describe a neuropsychological case report of a previously fit and well 54-year-old who acquired a range of severe and persisting neuropsychological impairments following a posterior reversible encephalopathy syndrome (PRES) complication of COVID-19. The initial clinical presentation included aphasia, a neurogenic foreign accent syndrome (FAS) and a persisting complete cortical blindness from the underpinning posterior parieto-occipital brain injury. Method: Neuropsychological single clinical case report. Results: The patient retained insight and made good early progress with their adjustment to the numerous losses as a result of the COVID-19 associated acquired brain injury. Comprehensive neuropsychological investigation characterised an acalculia, along with recorded deficits in focused, sustained and divided attention impacting on verbal memory registration and recall, information-processing speed, working memory and executive functioning, amongst numerous relative strengths. Conclusion: COVID-19 associated PRES, similar to PRES arising from other aetiologies, can in some cases result in irreversible acquired brain injury. The diverse neuropsychological effects need to be comprehensively investigated and managed in the pursuit of high quality patient care and neurorehabilitation. This case adds to the neuropsychological literature on PRES, FAS and COVID-19 associated acquired brain injury as a rare complication of SARS-CoV-2.
... Acute ischemia can be differentiated from PRES because it presents with cytotoxic edema and restricted diffusion. Fugate et al. suggested the following criteria for the diagnosis of PRES: neurological symptoms of acute onset, neuroimaging abnormalities of (focal) vasogenic edema, and the reversibility of clinical and/or radiological findings [7]. All cases were clearly distinguishable from acute ischemia, as there was no occlusion or stenosis in the vessels, especially those involving the PCA region, and angiogenic edema was seen in the bilateral frontal and occipital lobes. ...
Article
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Calcineurin inhibitors are essential immunosuppressive agents for kidney transplant patients. Posterior reversible leukoencephalopathy syndrome is a rare complication of treatment with calcineurin inhibitors. Herein, we report three cases of tacrolimus-associated posterior reversible leukoencephalopathy syndrome after kidney transplantation and their unique clinical courses. Patients in Cases 1 and 2 presented with headache and visual field disturbance on the fourth day after transplantation. Both patients were successfully treated with antihypertensive therapy and a change in medications from tacrolimus to cyclosporine and everolimus. Case 3 was a kidney transplant patient at 12 years post-transplant who developed fever and conjugate eye deviation to the right. The patient was initially treated with antihypertensive medication and a reduction in tacrolimus dose, but she experienced prolonged disturbance of consciousness, which eventually improved. These cases highlight the importance of considering the possibility of posterior reversible leukoencephalopathy syndrome in kidney transplant recipients taking calcineurin inhibitors who present with acute neurological dysfunction. They also demonstrate the need to perform appropriate imaging to confirm the diagnosis and initiate treatment, even if a significant amount of time has passed since transplantation. The introduction of an mTOR inhibitor may be practical when changing or discontinuing calcineurin inhibitors.
... The typical MRI finding is the symmetric vasogenic edema in the parietal and occipital lobes. However, up to the 27% of cases occur at the brainstem [2]. It is, therefore, crucial to bear PRES in mind even though the clinical imaging is atypical. ...
Preprint
A 71-year-old man had disordered consciousness whose Glasgow Coma Scale was E4V1M5. His blood pressure was high, but there was no abnormality in the cerebrospinal fluid examination. The MRI finding reveals a high-intensity area at the pons without the blood flow interruption. Thus, he has diagnosed with brainstem PRES
... PRES often presents non-specifically with symptoms manifesting over several hours or days. 1 Encephalopathy develops in 28%-94% of patients and ranges from mild confusion and cognitive deficits to stupor and sometimes coma. [1][2][3] Seizures affecting up to 74%-87% of patients 1 2 and typically occur within 24-48 hours of presentation 13 14 ; in a minority (3%-17%), seizures may evolve into status epilepticus, and rarely this is the presenting symptom. ...
Article
Posterior reversible encephalopathy syndrome (PRES) may present with diverse clinical symptoms including visual disturbance, headache, seizures and impaired consciousness. MRI shows oedema, usually involving the posterior subcortical regions. Triggering factors include hypertension, pre-eclampsia/eclampsia, renal failure, cytotoxic agents and autoimmune conditions. The mechanism underlying PRES is not certain, but endothelial dysfunction is implicated. Treatment is supportive and involves correcting the underlying cause and managing associated complications, such as seizures. Although most patients recover, PRES is not always reversible and may be associated with considerable morbidity and even mortality.
... Although not fully understood, endothelial dysfunction and abnormal cerebral blood flow were key factors in the pathophysiological changes underlying PRES (16,(24)(25)(26). MPA could induce inflammation within the vasculature of the CNS and result in ischemic or hemorrhagic damage to the brain parenchyma with resultant focal or generalized neurologic deficits (3)(4)(5)(6)(7)(8)(9). ...
Article
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Central nervous system (CNS) is rarely involved in microscopic polyangiitis (MPA). Here, we report a 14-year-old girl with MPA who developed new-onset seizures with deterioration of renal function. Her brain CT scan and MRI showed concurrent complications of intracerebral hemorrhage and posterior reversible encephalopathy syndrome (PRES). She got remission with combinations of methylprednisolone pulse, plasma exchange, regular hemodialysis, antiseizure and antihypertension medications. Furthermore, it is crucial to exclude the adverse effect of medications such as corticosteroid and biological therapy. We searched the literatures, retrieved 6 cases of MPA with PRES and summarized their clinical characteristics.
... The term "posterior reversible encephalopathy syndrome" (PRES) was first coined by Hinchey in 1996 (70) although the syndrome had been described earlier (71,72). It is a syndrome that usually presents early post HSCT (usually within the first 100 days (73) with a variety of clinical symptoms [seizures, headaches, hypertension, altered mentation, confusion and visual disturbance (74)(75)(76)(77) and distinct radiological features (vasogenic oedema of most commonly the parieto-occipital white matter but also the frontal and temporal lobes and posterior fossa] (78)(79)(80)(81)(82)(83). The incidence of PRES post HSCT is quite varied in the literature, ranging from 1.6 to 20% (9,84,85). ...
Article
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Despite advances in haematopoietic stem cell transplant (HSCT) techniques, the risk of serious side effects and complications still exists. Neurological complications, both acute and long term, are common following HSCT and contribute to significant morbidity and mortality. The aetiology of neurotoxicity includes infections and a wide variety of non-infectious causes such as drug toxicities, metabolic abnormalities, irradiation, vascular and immunologic events and the leukaemia itself. The majority of the literature on this subject is focussed on adults. The impact of the combination of neurotoxic drugs given before and during HSCT, radiotherapy and neurological complications on the developing and vulnerable paediatric and adolescent brain remains unclear. Moreover, the age-related sensitivity of the nervous system to toxic insults is still being investigated. In this article, we review current evidence regarding neurotoxicity following HSCT for acute lymphoblastic leukaemia in childhood. We focus on acute and long-term impacts. Understanding the aetiology and long-term sequelae of neurological complications in children is particularly important in the current era of immunotherapy for acute lymphoblastic leukaemia (such as chimeric antigen receptor T cells and bi-specific T-cell engager antibodies), which have well-known and common neurological side effects and may represent a future treatment modality for at least a fraction of HSCT-recipients.
... The parietal and occipital lobes are characteristically involved [3], with brainstem and spinal cord involvement reported only in atypical cases [4,5]. The common presenting complaints include seizures, headache, visual disturbances, and altered sensorium [6,7]. Diffusion-weighted MRI is the imaging modality of choice for confirming the presence of typical or atypical radiographic features [8,9]. ...
Article
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The rise in Coronavirus disease 2019 (COVID-19) cases is revealing its unique neurological manifestations. In light of the emerging evidence, a possible association with Posterior Reversible Encephalopathy Syndrome (PRES) is being consistently reported. We conducted a systematic literature search on four databases namely Pubmed/MEDLINE, Cochrane, Google Scholar, and Science Direct. After rigorous screening as per Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, a total of 34 articles describing 56 cases were selected as a part of this review. The mean age of the patients was 56.6 ± 15.3 years. The most common clinical presentation of PRES was altered mental status (58.9%) followed by seizures (46.4%) and visual disturbances (23.2%) while hypertension and diabetes mellitus were the most commonly reported comorbidities. 91.1% of the cases reported Magnetic Resonance Imaging (MRI) or Computed Tomography (CT) findings suggestive of PRES in the brain. Symptomatic management was employed in most of the cases to control seizures and blood pressure, and 44 patients (78.5%) fully or partially recovered. The most likely underlying mechanism involves COVID-19 mediated cytokine storm syndrome that leads to endothelial damage and increased permeability of the cerebral vessels, thus causing the characteristic edema of PRES. High neuronal and glial cell expression of Angiotensin Converting Enzyme-2 (ACE-2) receptors also suggests the possibility of direct viral damage. Since timely diagnosis and treatment reports a good prognosis, it is vital for physicians and neurologists to be well-versed with this association.
... Immunosuppressive or immunomodulatory therapies have also been in implicated in the causation of PRES. In a large case series, it was shown that half of the patients with PRES had a history of immunosuppressive medication intake [13]. There is no direct connection between the serum levels of immunosuppressive medication and the development of PRES [14]. ...
Article
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BACKGROUND K2 is an artificially synthesized cannabinoid (SCB), manufactured as a non-consumption herbal incense but increasingly misused as a recreational drug. Posterior reversible encephalopathy syndrome (PRES) is a rare clinical and radiological entity characterized by brain edema, often in the setting of acute hypertension. Cases of PRES caused by recreational drug use have been reported in the literature. CASE REPORT We report an unusual case of PRES after consumption of K2 in a 24-year-old healthy woman who presented with episodic agitation and altered mental status. Magnetic resonance imaging showed nonspecific subtle high T2/FLAIR (fluid-attenuated inversion recovery) signal intensities in the region of the posterior parietal and occipital cortices. Her extensive drug screen report was positive for K2. Her mental status improved over the course of 3 weeks and she had returned to her baseline at 3-month follow-up. CONCLUSIONS Our case highlights the importance of having a high clinical suspicion in patients presenting with altered mental status and a history of recreational drug use. K2 is not detected by routine urine drug testing, so a high level of clinical suspicion is required to request an extensive drug screen. It is important for the physician to counsel active synthetic cannabinoid users regarding these rare complications.
... Autoimmune disease is considered to cause PRES by mechanisms of both vasogenic edema and endothelial dysfunction (13). Interestingly, patients with autoimmune disease were reported to be more likely to have cerebellar involvement (14), as in our case, than others. There was a small number of cases of AAV complicated by PRES. ...
Article
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A young woman with microscopic polyangiitis (MPA) requiring hemodialysis showed repeated posterior reversible encephalopathy syndrome (PRES) with spatiotemporal multiple lesions over a period of two months. The first PRES episode with confusion and the second PRES episode with vertigo and nausea were caused by MPA, hypertension and renal failure. These symptoms were improved by the reinforcement of MPA treatment and blood pressure management. The third PRES episode with nausea, headache, seizure and visual changes was induced by rituximab infusion and hypertension. The PRES was improved with blood pressure and convulsant management. These conditions are challenging to diagnose and treat.
... As reported by Pilato and colleagues, the presence of a systemic immune impairment should be considered in the pathogenesis of PRES, especially in normotensive patients; this possibility is supported by the fact that both our patients were suffering from GBS, which is strongly associated with a dysregulation of immune system response (3). In addition, the hypothesis of an altered endothelial function has been supposed, especially in patients with autoimmune diseases (10). ...
Article
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Background Posterior reversible encephalopathy syndrome (PRES), reversible cerebral vasoconstriction syndrome (RCVS), or the coexistence of these two entities shares similar risk factors and clinical features. For these conditions, a common origin has been supposed. Even if the majority of patients show a favorable course and a good prognosis, a small percentage of cases develop neurological complications. Up to date, only about 30 cases of PRES associated with Guillain-Barré syndrome (GBS) have been reported in the literature. Cases Here, we present two cases of a particularly aggressive PRES/RCVS overlap syndrome, associated with acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyneuropathy (AIDP) variants of GBS, respectively, presenting with similar initial clinical aspects and developing both an atypical and unfavorable outcome. On MRI examination, the first patient showed typical aspects of PRES, while, in the second case, radiological features were atypical and characterized by diffusion restriction on the apparent diffusion coefficient (ADC) map. The first patient demonstrated rapid worsening of clinical conditions until death; the second one manifested and maintained neurological deficits with a permanent disability. Conclusions We suggest that PRES may conceal RCVS aspects, especially in most severe cases or when associated with a dysimmune syndrome in which autoimmune system and endothelial dysfunction probably play a prominent role in the pathogenesis. Although the role of IVIg treatment in the pathogenesis of PRES has been proposed, we suggest that GBS itself should be considered an independent risk factor in developing PRES.
... It is usually seen in association with malignant hypertension, eclampsia. Typical MRI brain findings are of bilateral white-matter abnormalities in vascular watershed areas affecting mostly the occipital and parietal lobes [16,17,18]. With proper management of hypertension and other associated risk factors, PRES can be completely reversed including the MRI findings. ...
... Magnetic resonance imaging (MRI) could be used as a non-radiation and non-invasive method for evaluation of the brain lesions. Previous MRI studies on PE mostly focused on posterior reversible encephalopathy syndrome (PRES) (Fugate et al., 2010). However, PRES has a wide disease spectrum, namely, hypertension, sepsis, nephropathy, and the patients using immunosuppressive agents (Dong et al., 2017). ...
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Background Pre-eclampsia (PE) is the main cause of death in maternal and prenatal morbidity. No effective clinical tools could be used for the prediction of PE. A radiomics nomogram based on diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) maps was established to predict PE from gestational hypertension (GH). Materials and methods A total of 138 patients with hypertensive disorders of pregnancy were continuously enrolled in the study prospectively, namely, 58 patients with PE and 80 patients with GH. The patients were randomly divided into a training cohort ( n = 97) and a test cohort ( n = 41). Radiomics features were extracted from DWI and ADC maps. The radiomics signature was constructed using a least absolute shrinkage and selection operator (LASSO) algorithm in the training cohort. A radiomics nomogram was developed by combining the radiomics signature with the selected clinical risk factors. The area under the receiver operating characteristic (ROC) curves (AUC), specificity, sensitivity, accuracy, positive predictive value, and negative predictive values of the radiomics signature, clinical risk factors, and radiomics nomogram were calculated. Decision curve analysis (DCA) was performed to determine the clinical usefulness of the radiomics nomogram. Results The LASSO analysis finally included 11 radiomics features, which were defined as the radiomics signature. The individualized prediction nomogram was constructed by integrating the radiomics signature, maternal age, and body mass index (BMI). The nomogram exhibited a good performance both in the training cohort [AUC of 0.89 (95% CI, 0.82–0.95)] and test cohort [AUC of 0.85 (95% CI, 0.73–0.97)] for predicting PE from GH. The DCA indicated that clinicians and patients could benefit from the use of radiomics nomogram. Conclusion The radiomics nomogram could individually predict PE from GH. The nomogram could be conveniently used to facilitate the treatment decision for clinicians and patients.
... However, we now know that up to 30% of patients with PRES do not have the high blood pressures necessary to exceed the autoregulatory control of the cerebral vasculature. Here, endothelial dysfunction would be the main culprit, which in turn could be caused by various toxins, immunosuppressive drugs, chemotherapy or septic conditions, among others [35][36][37][38][39]. In PRES, T2WI and T2 FLAIR sequences are probably the most useful, given their sensitivity for detecting vasogenic edema on MRI, as well as DWI sequences and the ADC map. ...
Article
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Central nervous system involvement related to decompression sickness (DCS) is a very rare complication of breath-hold diving. So far, it has been postulated that repeated dives with short surface intervals represent a key factor in the development of breath-holding-related DCS. We report the case of a breath-hold diver who, after repeated immersion, developed DCS with brain involvement. After treatment in a hyperbaric chamber, there was a clinical improvement in the symptoms. Magnetic resonance imaging of the brain showed hyperintense lesions in long-time repetition sequences (FLAIR, T2WI) in the left frontal and right temporal lobes. Diffusion-weighted imaging (DWI) sequences and the apparent diffusion coefficient (ADC) map were characteristic of vasogenic edema, allowing us to exclude the ischemic nature of the process. These findings, together with the acute clinical presentation, the resolution of lesions in evolutionary radiological controls and the possible involvement of blood-brain barrier/endothelial dysfunction in DCS, could suggest a new form of posterior reversible encephalopathy syndrome (PRES)-like presentation of DCS. This would represent a novel mechanism to explain the pathophysiology of this entity. We conducted a literature review, analyzing the pathophysiological and neuroimaging characteristics of DCS in breath-hold diving based on a case of this rare disease.
... It is an acute neurological disorder that usually occurs with hypertension, end-stage renal disease, active cancer, chemotherapy, allogenic bone marrow transplantation, autoimmune disorders, preeclampsia or eclampsia, and sepsis [2,3]. Although the exact etiopathogenesis leading to PRES in the various predisposing conditions remains unknown, postulated hypotheses include vasogenic, neuropeptide, cytotoxic, and immunogenic theories that result in endothelial injury, disruption of the blood-brain barrier, and eventual cerebral edema [4][5][6][7]. ...
Article
Purpose Assess factors in posterior reversible encephalopathy syndrome (PRES) related to intensive care unit (ICU) admissions , mechanical ventilation, and length of stay (LoS). Methods Retrospectively, we collected clinical, biochemical, and imaging data of PRES patients. MRI studies were assessed for imaging severity, and complications, including restricted diffusion and hemorrhage. Univariate and multivariate regression analyses were performed for factors associated with ICU admission, mechanical ventilation, and LoS. We assessed for association between clinical and biochemical factors and imaging severity grading systems and complications. Results We had 57 subjects with mean ± SD age of 56.3 ± 14.5 years and 68.3% were females. In 60 cases, 36.7% had hyper-tension, 23.3% had chronic renal disease, 18.3% had sepsis, 16.7% were on active chemotherapy, 10% underwent hematopoi-etic stem cell transplantation (HSCT), 10% with active cancer, 6.7% were eclampsia/preeclampsia, and 1.7% had radiotherapy. We had 17 (26.6%) grade 1, 26 (46.8%) grade 2, 17 (26.6%) grade 3 PRES based on vasogenic edema extent, and 28 (46.7%) severe PRES (≥ 5 areas) cases. 19 (31.7%) had restricted diffusion with hemorrhage in 19 (31.7%) cases. On multivariate analysis, ICU admissions showed significant association with hypertension (OR = 5.57, 95% CI: 0.96-32.23; p = 0.05), and raised INR (OR = 119, 95% CI: 1.1-1244.3; p = 0.04); LoS with HSCT, mean arterial pressure (MAP), intraparenchymal hematoma, and sepsis; and extent of vasogenic edema with restricted diffusion (OR = 4.31, 95% CI: 1.5-12.33; p < 0.01). Conclusion In summary, PRES with hypertension and elevated INR are associated with ICU admissions. HSCT, MAP, intraparenchymal hematoma, and sepsis correlated with LoS. Imaging severity grading based on vasogenic edema extent may be better due to association with restricted diffusion.
... The auto-regulating response is insufficient when blood pressure rises rapidly and dramatically, resulting in hyperperfusion and extravasation of plasma and macromolecules. The posterior circulation of the brain is comparably less innervated with sympathetic nerves, which is most likely the cause of PRES's preferential involvement of the posterior portion of the brain [7]. ...
... We learned from this case that CMT 2N can also mimic stroke and present similar imaging changes. The diagnostic criteria for PRES include neurological symptoms of acute attack, brain imaging abnormalities suggestive of vasogenic (focal) edema, and the reversibility of clinical and/or radiological findings (27). Neuroimaging reveals characteristic findings: symmetrical focal T2-hyperintensities mainly in the parietal and occipital lobes, followed by the frontal lobes and the cerebellum (28). ...
Article
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Introduction Charcot-Marie-Tooth (CMT) disease is a rare group of peripheral neuropathies with high clinical and genetic heterogeneity. CMT type 2N (CMT 2N) is a rare subtype of CMT with few clinical reports. The clinical presentation mimics that of other diseases, frequently leading to misdiagnoses. We present a case of CMT 2N with reversible white matter lesions (WMLs), which repeatedly mimicked stroke or encephalitis. We include a literature review to the improve management of this disease. Case Description An 8-year-old boy was admitted to the hospital with slurred speech and limb weakness that had persisted for 1 day. Physical examination revealed lethargy, dysarthria, and a positive bilateral Babinski sign. Cerebrospinal fluid (CSF) analysis showed no abnormalities. Brain magnetic resonance imaging (MRI) revealed symmetrical abnormal signal areas in the paraventricular white matter and corpus callosum. The patient was suspected of having viral encephalitis and recovered rapidly after treatment. He was hospitalized 3 years later for limb weakness, barylalia, and facial paralysis persisting for 1 day. MRI showed an abnormal signal in the bilateral corona radiata. He was suspected of having a stroke or encephalitis. He was completely recovered after treatment. After a second 3-year span, he was admitted for another stroke-like episode. Physical examination revealed facial-lingual hemiparesis, mild atrophy of the left thenar muscle, decreased muscle strength in the extremities, and disappearance of the tendon reflex. MRI revealed more pronounced abnormal signals in the bilateral centrum semiovale and corpus callosum. Antibodies against autoimmune encephalitis were negative. A nerve conduction velocity (NCV) study showed motor and sensory four-limb nerve demyelination with axonal damage, most notably at the distal end. His symptoms were resolved after active treatment. A follow-up MRI showed the complete disappearance of the abnormal white matter signal. Whole exon sequencing showed a heterozygous mutation [c.2093C > T(p.Ser698Phe)] in the alanyl-tRNA synthetase 1 gene ( AARS1 ). His mutation, clinical features, and electrophysiological testing led to a diagnosis of CMT 2N. Discussion Early-Onset CMT 2N with reversible WMLs can often mimic stroke or encephalopathy. Affected individuals may show an atypical posterior reversible encephalopathy syndrome (PRES) on MRI. Careful family history assessment, physical examination, nerve conduction studies, MRIs, and genetic testing are essential for early diagnosis. Further studies are required to confirm these findings.
... In adults, the slightly higher predominance in female population compared to male is still to be clarified. 10 Etiological aspects may account for this finding, since women are more prone to acute inflammation disorders, 11 which are more frequent in adults as compared to children (45% on average in adults vs 14.3% in our review). 12 It is hypothesized that children have a narrower range of cerebral autoregulation and therefore the probability of having PRES is even higher than that in adults, in situations such as acute rise in blood pressure. ...
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Although more commonly seen in adult population, posterior reversible encephalopathy syndrome (PRES) can also be observed in pediatric patients. The etiopathogenesis of pediatric PRES is poorly understood, and the available evidence comes mostly from childhood cancer. Analysis of the sociodemographic and clinical characteristics of the different noncancer-related types can improve the understanding of pediatric PRES. Methods: Systematic review of characteristics and outcome of noncancer pediatric PRES. Primary sources of investigation were identified and selected through Pubmed and Scopus databases. The research was complemented by reference search in relevant publications. Study protocol was registered (Prospero CRD42020165798). Results: We identified 449 cases of noncancer pediatric PRES from 272 studies, median age 10 (newborn to 17 years), 49.9% girls. The 4 most common groups of conditions were renal 165 (36.7%), hematologic 84 (18.7%), autoimmune 64 (14.3%), and cardiovascular 28 (6.2%) disorders. The 4 most prevalent precipitants identified were hypertensive crisis 119 (26.5%), corticosteroids 56 (12.5%), immunosuppression drugs 44 (9.8%), and biologic drugs 14 (3.1%). Clinical presentations included seizures 100 (22.3%), headaches 22 (4.9%), encephalopathy 17 (3.8%), visual disturbances 6 (1.3%), and focal deficit 3 (0.7%). The distribution of lesions was (n = 380): combined anterior/posterior circulation (40.8%), isolated posterior circulation (24.1%), anterior circulation (6.2%), and deep structures (1.6%). Residual neurological deficits occurred in about 1 out 10 cases. Conclusion: Pediatric PRES differs from the adult in etiology, precipitants, and clinical manifestations. Renal diseases predominate, acute raised blood pressure is less frequent, and cortical deficits are rarer. In addition, the proportion of patients with combined anterior/posterior circulation was higher. Permanent neurological sequels can occur.
... It is a reversible disorder of white matter of brain with rapid-onset symptoms like headache, seizures, altered mental status, and visual blurring. It is commonly associated with hypertension, renal failure, immunosuppressive agents, and autoimmune disorders [2,3]. It may also occur in patients with hematological disorders receiving cytotoxic drugs and immunosuppressive agents and in patients undergoing hematopoietic stem cell transplant (HSCT) [4][5][6]. ...
Article
A 53-year-old woman with a 6-year history of rheumatoid arthritis (RA) presented with pharyngeal pain, fever, and altered mental status. The patient had been treated with methotrexate (MTX) 12 mg/week, baricitinib 4 mg/day, and tacrolimus 2 mg/day. Magnetic resonance imaging of the brain revealed diffuse high-intensity lesions in the cerebral white matter, basal ganglia, brainstem, and right cerebellar hemisphere. She was diagnosed with Epstein-Barr virus (EBV) encephalitis due to elevated levels of EBV-DNA in the cerebrospinal fluid and serum. Although MTX-associated lymphoproliferative disorders are well-known complications in patients with RA, EBV encephalitis requires careful attention for such patients undergoing treatment with multiple potent immunosuppressants.
Article
Background Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic disorder characterized by seizures, headache, altered mental status, and visual disturbances, and is often associated with acute hypertension. Objective This narrative review provides a focused description of the presentation, diagnostic evaluation, and management of PRES. Discussion PRES is associated with a variety of factors, including acute rise in blood pressure, renal disease, preeclampsia/eclampsia, and immunosuppressive therapy. The pathophysiology is theorized to involve dysfunction of cerebral autoregulation leading to vascular leak or endothelial dysfunction resulting in vasogenic edema. In the emergency department (ED), clinical findings suggestive of PRES should prompt diagnostic testing focused on confirming the diagnosis and excluding other conditions that may present similarly. Laboratory studies are primarily useful for excluding alternative diagnoses. Computed tomography (CT) and, in particular, magnetic resonance imaging (MRI) are the recommended neuroimaging modalities for diagnosis. CT and MRI may demonstrate cerebral vasogenic edema, most often in the distribution of the posterior circulation. Treatment involves management of seizures, control of blood pressure if elevated, and treatment of any underlying trigger. Conclusion PRES is a neurological disorder that is typically reversible if recognized on presentation and promptly and appropriately managed. This narrative review characterizes this condition for emergency clinicians.
Article
Objectives: We aimed to identify independent predictors of the clinical outcomes of posterior reversible encephalopathy syndrome (PRES) in the Thai-Asian population. Materials and methods: This retrospective cohort study recruited PRES patients admitted in the hospital between 2006 and 2019. The baseline characteristics, clinical presentations, precipitating factors, neuroimaging characteristics, hospital complications, and hospital outcomes were compared between the groups with favourable (modified Rankin scale [mRS] score, 0-2) and unfavourable (mRS score, 3-6) clinical outcomes. Factors presenting with p-values < 0.5 in univariate analysis were analysed in the multiple logistic regression model to determine independent predictors of outcome. Results: Among 136 PRES patients, 22.80% experienced unfavourable outcomes (mRS score, 3-6), and the mortality rate was 7.35%. The common presenting symptoms in the unfavourable clinical outcome group were impairment of consciousness (90.32%) and seizures (67.74%). Sepsis as a hospital complication (adjusted odds ratio [aOR], 32.95; 95% confidence interval [CI], 4.44-244.22, p = 0.001), acute kidney injury as a hospital complication (aOR, 9.94; 95% CI, 1.71-57.66; p = 0.010), and impairment of consciousness (aOR, 10.85; 95% CI, 1.72-68.53, p = 0.011) were independent predictors of unfavourable outcomes. On the other hand, headache was an independent protective factor (aOR, 0.164; 95% CI, 0.03-0.91; p = 0.039). Conclusions: Impairment of consciousness, sepsis as a hospital complication, and acute kidney injury were independent predictors of unfavourable clinical outcomes, whereas headache showed a preventive effect.
Article
Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder characterized by headache, seizures, confusion and visual disturbances, as well as potentially reversible neuroimaging findings in most patients after proper treatment. Seizures is one of the most common clinical presentations of PRES. This review summarizes the potential pathophysiology and clinical features of PRES, as well as a multimodal approach to imaging and also briefly discusses the phenomenon of seizures in paediatric population.
Article
Introduction. The syndrome of posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological condition caused by vasogenic brain edema due to elevated blood pressure. The development of this syndrome is associated with the toxic effect of drugs in the treatment of oncological diseases. In most cases, timely diagnosis and therapy of PRES determine the complete reversibility of symptoms. However, a poor prognosis is possible with the development of severe disabling consequences or death. In practice, there are difficulties in the diagnosis of PRES due to the lack of specificity of clinical manifestations and awareness of many doctors about the existence of this syndrome. The aim of the study was to present the clinical-radiological aspect of the syndrome of posterior reversible encephalopathy in children with oncological diseases. Materials and methods. The analysis of 8 medical histories of children with oncological pathology complicated by the syndrome of posterior reversible encephalopathy was carried out. Results. The study group was dominated by patients with hemato-oncological diseases. The development of PRES occurred during chemotherapy and in most cases was not associated with arterial hypertension. The clinical picture was dominated by such symptoms as impairment of consciousness, seizures and movement disorders in the form of paresis. When carrying out magnetic resonance imaging (MRI), the lesion of the white matter of the brain was determined, mainly in the projection of the parieto-occipital regions. During treatment, all patients showed reversibility of neurological symptoms with leveling of radiological signs. Discussion. When analyzing the clinical and radiological characteristics of PRES in children, a comparison of the data with clinical observations presented in the literature was noted. The role of chemotherapy as a triggering factor in the development of posterior reversible encephalopathy syndrome has been determined. Conclusion. The necessity of including the syndrome of posterior reversible encephalopathy in the list of differential diagnoses in patients receiving chemotherapy is shown.
Article
Posterior reversible encephalopathy syndrome (PRES) is a neurological condition with a wide range of symptoms, including visual disturbances, headache, vomiting, seizures, and altered consciousness. This review describes the pathophysiology of PRES, as well as the clinical, diagnostic, and therapeutic intervention during pregnancy. The gold standard for diagnosis of PRES is Magnetic Resonance Imaging (MRI), helping to differentiate it from other similar conditions. The aim of this paper is to review the principal aspects of PRES, general care, blood pressure control, and seizures prevention while avoiding potential injuries to the mother and fetus in the event of pregnancy. We concluded that PRES can be effectively treated and reversed if prompt diagnostic action is made, and adequate care is initiated.
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Vascular disease affects many different arterial beds throughout the body. Yet the brain is susceptible to several vascular disorders that either are not found in other parts of the body or when found are much less likely to cause clinical syndromes in other organs. This specific vulnerability of the brain may be explained by structural and functional differences between the vessels of the brain and those of vessels in other parts of the body. In this review, we focus on how cerebrovascular anatomy and physiology may make the brain and its vessels more susceptible to unique vascular pathologies. To highlight these differences, we use our knowledge of five diseases and syndromes that most commonly manifest in the intracranial vasculature. For each, we identify characteristics of the intracranial arteries that make them susceptible to these diseases, while noting areas of uncertainty requiring further research.
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Thrombotic microangiopathy is characterised by endothelial cell injury, intravascular platelet-fibrin thrombi, and vascular damage, leading to acute kidney injury, thrombocytopenia, and microangiopathic haemolytic anaemia. Among the autoimmune diseases related to thrombotic microangiopathy, anti-neutrophil cytoplasmic antibody-associated vasculitis-related thrombotic microangiopathy cases have been rarely reported; therefore, the optimal treatment for associated vasculitis-related thrombotic microangiopathy remains unknown. An 84-year-old woman without significant medical history presented with a 1-month history of general fatigue, fever, and deteriorating bilateral leg numbness and was admitted to our hospital. She had elevated myeloperoxidase anti-neutrophil cytoplasmic antibody levels, polyneuropathy, and rapid progressive glomerulonephritis because of pauci-immune crescentic glomerulonephritis, as revealed by a kidney biopsy. Accordingly, we diagnosed her with microscopic polyangiitis. After administering methylprednisolone pulse therapy, rituximab, and intravenous immunoglobulin, the patient’s mental state deteriorated, presenting signs of thrombotic microangiopathy with posterior reversible encephalopathy syndrome. Intermittent haemodialysis and plasma exchange were initiated; however, her condition was not improved, and eculizumab administration was initiated thereafter. The patient’s symptoms showed a remarkable response to eculizumab; thrombotic microangiopathy findings, kidney function, and neurological symptoms improved after only two doses of eculizumab, and she achieved sustained remission. The extremely effective course of eculizumab treatment indicated that overt complement activation affected the development of thrombotic microangiopathy. Anti-neutrophil cytoplasmic antibody-associated vasculitis-related thrombotic microangiopathy may be mediated by complement activation, and prompt induction of eculizumab therapy may be a superior strategy to prevent organ damage. Further studies should elucidate the role of complement activation in associated vasculitis-related thrombotic microangiopathy and the efficacy of eculizumab treatment.
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A 71‐year‐old man had disordered consciousness whose Glasgow Coma Scale was E4V1M5. His blood pressure was high, but there was no abnormality in the cerebrospinal fluid examination. The MRI finding reveals a high‐intensity area at the pons without the blood flow interruption. Thus, he has diagnosed with brainstem PRES. The typical MRI finding of PRES is the presence of a symmetric vasogenic edema in the parietal and occipital lobes. However, up to the 27% of cases occur at the brainstem, and it is imperative to remember this less frequent localization.
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Magnetic resonance imaging perfusion (MRP) techniques can improve the selection of acute ischemic stroke patients for treatment by estimating the salvageable area of decreased perfusion, that is, penumbra. Arterial spin labeling (ASL) is a noncontrast MRP technique that is used to assess cerebral blood flow without the use of intravenous gadolinium contrast. Thus, ASL is of particular interest in stroke imaging. This article will review clinical applications of ASL in stroke such as assessment of the core infarct and penumbra, localization of the vascular occlusion, and collateral status. Given the nonspecific symptoms that patients can present with, differentiating between stroke and a stroke mimic is a diagnostic dilemma. ASL not only helps in differentiating stroke from stroke mimic but also can be used to specify the exact mimic when used in conjunction with the symptomatology and structural imaging. In addition to a case-based overview of clinical applications of the ASL in stroke and stroke mimics in this article, the more commonly used ASL labeling techniques as well as emerging ASL techniques, future developments, and limitations will be reviewed.
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Background: Sepsis-associated encephalopathy (SAE) is frequently encountered in sepsis and is often accompanied by neuroimaging findings indicating ischemia, hemorrhage, and edema. Posterior reversible encephalopathy syndrome (PRES) has been vastly underrecognized in previously reported cohorts of patients with sepsis and SAE. Our aim was to determine the prevalence and distinguishing clinical, neuroimaging, and electroencephalography features of PRES in SAE. Methods: In this prospective observational study, patients with radiologically identified PRES were selected from a consecutively enrolled cohort of 156 patients with SAE and assessed for neurological outcome using the extended Glasgow Outcome Scale for 12 months. Patients with SAE and PRES and other types of brain lesions were compared in terms of clinical and diagnostic workup features. Results: Fourteen of 156 patients (8.9%) were determined to be radiologically compatible with PRES, whereas 48 patients displayed other types of acute brain lesions. Patients with PRES often showed lesions in atypical regions, including frontal lobes, the corpus callosum, and the basal ganglia. Source of infection was mostly gram-negative bacteria originating from pneumonia or intraabdominal infections. Patients with PRES were not different from other patients with SAE with brain lesions in terms of features of sepsis and neurological outcome. However, patients with PRES showed increased prevalence of seizures and intraabdominal source of infection. Conclusions: PRES is highly prevalent in SAE, often encompasses unusual brain regions, and usually presents with generalized seizures. Patients with SAE and PRES do not appear to have distinguishing clinical and diagnostic workup features. However, generalized seizures may serve as warning signs for presence of PRES in patients with SAE.
Article
A woman in her 60s developed acute onset headache, blurry vision and encephalopathy a few hours after rituximab infusion, given to treat granulomatosis with polyangiitis. CT scan showed oedema in the posterior circulation area suggesting the diagnosis of posterior reversible encephalopathy syndrome, and an MRI confirmed it. After being treated with aggressive blood pressure control and other supportive measures, her symptoms improved over 3-4 days. This case highlights the need for awareness and early recognition of this rare but serious adverse effect of rituximab. CT scan can be helpful in diagnosis (also to rule out bleeding), but the MRI provides the most accurate diagnosis.
Article
Background: We aimed to investigate the clinical features and prognosis of posterior reversible encephalopathy syndrome (PRES) in children. Methods: Clinical data of children with PRES diagnosed at the Children's Hospital of Chongqing Medical University from June 2015 to May 2019 were retrospectively analyzed. Results: The study enrolled 47 patients with a mean age at diagnosis of 8.79 ± 3.72 years (range, 2-15 years). PRES causes included renal disorder (29/47), hematological disease (13/47), and hypertension (5/47). PRES manifested as seizure (43/47), headache (28/47), visual impairment (18/47), dizziness (18/47), vomiting (18/47), and mental and behavioral abnormalities (17/47). Forty-six children had hypertension (46/47) at PRES onset. Magnetic resonance imaging (MRI) mainly involved the parietal and occipital lobes (42/47), 38 cases were mild (38/47), 7 were moderate (7/47), and 2 were severe (2/47). The clinical symptoms of 41 patients (41/47) were relieved within 1-2 weeks. Thirty-seven children were followed up for 7-54 months (modified Rankin Scale). Twenty-five children had favorable outcomes (25/37). Twelve children had adverse outcomes (12/37), including epilepsy, disorders of consciousness, visual impairment, and mental decline. Analysis of single factors revealed that severity on MRI, length of in-hospital stay, and mental and behavioral abnormalities were related to adverse outcomes after PRES. Analysis of multiple factors revealed that severity on MRI and length of in-hospital stay were independent risk factors for PRES. Conclusion: Pediatric PRES is a clinical radiographic syndrome with multiple etiologies. Most patients have a good prognosis. Severity on MRI and length of in-hospital stay are independent risk factors.
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Hematologic disorders and malignancies are commonly encountered in children. The central nervous system is affected by many benign and malignant hematological diseases. Neurologic findings may occur due to central involvement of hematological disease or neurotoxic effects of treatment during the disease. At all these stages, radiological imaging and evaluation have a critical role. In this review article, we aim to describe the imaging findings of central nervous system involvement in pediatric hematologic diseases. This review was prepared based on the latest literature available in the PubMed database in the English language from inception to March 2022. The radiological images of the patients in our archive were obtained from the PACS (Picture Archiving and Communication Systems) to set an example for the diseases described in the text.
Objectives To report a case of a patient with overlapping posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS), and review the existing literature emphasizing the pathophysiological overlap of these two entities. Materials and Methods We conducted a literature search in electronic database PubMed identifying studies reporting the overlap of PRES and RCVS. Results PRES and RCVS are two increasingly recognized entities that share similar clinical and imaging features. PRES is characterized by vasogenic edema predominantly in the parieto-occipital regions, associated with acute onset of neurological symptoms including encephalopathy, seizures, headaches, and visual disturbances. RCVS is characterized by reversible segmental and multifocal vasoconstriction of the cerebral arteries and classically presents with thunderclap headache, with or without associated focal neurological deficits and seizures. PRES is frequently associated with uncontrolled hypertension but can also be seen in the setting of renal failure, exposure to cytotoxic agents, or pre-eclampsia. RCVS is often triggered by exposure to vasoactive agents, postpartum state, or immunosuppression. We report a case of a patient presenting with vision changes and hemiparesis, and found to have extensive cytotoxic and vasogenic edema involving the cortex and subcortical white matter on brain imaging. These changes were primarily noted in the parieto-occipital and brainstem regions, along with features of reversible vasculopathy on vascular imaging suggestive of coexisting PRES and RCVS. Conclusions PRES and RCVS share precipitating factors, clinical and radiological features, and frequently co-exist, suggesting a common pathophysiological mechanism related to reversible dysregulation of cerebral vasculature, endothelial dysfunction, and breakdown of the blood-brain barrier.
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Computed tomographic (CT) and magnetic resonance imaging (MRI) views of the brain were obtained in two adults and one child with hypertensive encephalopathy (HTE). Noncontrast CT was normal in one case and demonstrated decreased density posteriorly in two cases; MRI demonstrated focal, symmetric increased signal intensity in white matter and cortex, with occipital lobe involvement in each case. These lesions were better visualized on T2-weighted than on spin density images and were resolved on follow-up MRI four to five weeks later. These MRI studies support the concept that HTE is caused by the multifocal extravasation of fluid and proteins across the blood-brain barrier during "breakthrough" of cerebral autoregulation. We found that MRI appears more sensitive than CT and better defines the anatomy of cerebral involvement in HTE.
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Standard MRI confirms the diagnosis of posterior leukoencephalopathy syndrome (PLES), recently associated with an increasing number of medical conditions. In PLES, T2-weighted MRI demonstrates hyperintensity spreading out from posterior brain regions; the pathophysiology remains mysterious. In the acute setting, diffusion-weighted imaging (DWI), but not standard MR imaging, can distinguish ischemic injury from those conditions known to cause vasogenic brain edema. DWI is potentially valuable in understanding the pathophysiology of PLES and in diagnosing patients who do not have previously known risk factors. Serial CT and MRI studies (including DWI, apparent diffusion coefficient [ADC] maps, and, in one instance, perfusion-weighted imaging) were performed in three female patients with a neurologic syndrome consistent with PLES while hospitalized for treatment of other conditions. None of the patients had previously described risk factors for PLES; all had only mild elevations in blood pressure. MRI showed large, abnormal, T2 hyperintense regions in the posterior cerebrum with corresponding hyperintensity on ADC maps-signal characteristics predominantly consistent with vasogenic edema. There were also smaller patchy posterior cortical regions with decreased ADC and bright DWI consistent with infarction in one, and dramatic conversion of a large region to an ischemic pattern in another. ADC maps and DWI can successfully differentiate PLES from early cerebral ischemia, thus playing a pivotal role in treatment decisions. PLES is associated with a wider variety of conditions than has been previously reported and is not always reversible. Hyperintense DWI signal in patients with the syndrome likely marks a tissue stage of permanent brain injury.
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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. 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. 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.
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Encephalopathy associated with autoimmune thyroid disease is an underdiagnosed clinical condition. No specific disease marker exists and to date, imaging techniques have not proven useful. The disease is characterized by seizures, stroke-like events and psychiatric symptoms. Here, we present the report of a patient with encephalopathy associated with autoimmune thyroid disease in which a reversible white matter disorder was detected by MR imaging. This encephalopathy was alleviated and later disappeared following corticosteroid treatment. We conclude that the white matter is the principal brain tissue involved in this condition and that brain MRI studies may prove to be a useful tool in the diagnosis of patients suffering this treatable disease.
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The mechanisms responsible for cyclosporin-induced encephalopathy remain controversial. Herein we present a case of cyclosporin-induced encephalopathy with unusually prolonged vasospasm, which might have contributed to the slow recovery of the patient.
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The cause of "posterior reversible encephalopathy syndrome" (PRES) is not established. We recently encountered several patients who developed PRES in the setting of severe infection. In this study, we comprehensively reviewed the clinical and imaging features in a large cohort of patients who developed PRES, with particular attention to those with isolated infection, sepsis, or shock (I/S/S). The clinical/imaging features of 106 patients who developed PRES were comprehensively evaluated. In 25 of these patients, PRES occurred in association with severe I/S/S separate from transplantation. The clinical/imaging features (computer tomography, MR imaging, and MR angiography [MRA]) of the patients with I/S/S were further evaluated, including organ/tissue/blood culture results, mean arterial blood pressure (MAP) at toxicity, extent of cerebral edema, and presence of vasospasm. PRES occurred in association with I/S/S in 25 of 106 patients (23.6%), in addition to 4 other major clinical settings, including cyclosporine/FK-506 (post-transplant) neurotoxicity (46.2%), autoimmune disease (10.4%), postchemotherapy (3.7%), and eclampsia (10.4%). In the 25 patients with I/S/S, available cultures demonstrated a predominance of gram-positive organisms (84%). Blood pressure was "normal" at toxicity in 10 patients (MAP, 95 mm Hg); "severe" hypertension was present in 15 patients (MAP, 137 mm Hg). Extent of brain edema graded on imaging studies was greater in the normal MAP group compared with the severe hypertension group (P < .05). MRA demonstrated vasospasm in patients with severe hypertension and vessel "pruning" in the normal MAP group. Infection/sepsis/shock may be an important cause of PRES, particularly in relation to infection with gram-positive organisms.
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To examine predictors and the prognostic value of electrographic seizures (ESZs) and periodic epileptiform discharges (PEDs) in medical intensive care unit (MICU) patients without a primary acute neurologic condition. Retrospective study. MICU in a university hospital. A total of 201 consecutive patients admitted to the MICU between July 2004 and January 2007 without known acute neurologic injury and who underwent continuous electroencephalography monitoring (cEEG) for investigation of possible seizures or changes in mental status. None. Median time from intensive care unit (ICU) admission to cEEG was 1 day (interquartile range 1-4). The majority of patients (60%) had sepsis as the primary admission diagnosis and 48% were comatose at the time of cEEG. Ten percent (n = 21) of patients had ESZs, 17% (n = 34) had PEDs, 5% (n = 10) had both, and 22% (n = 45) had either ESZs or PEDs. Seizures during cEEG were purely electrographic (no detectable clinical correlate) in the majority (67%) of patients. Patients with sepsis had a higher rate of ESZs or PEDs than those without sepsis (32% vs. 9%, p < 0.001). On multivariable analysis, sepsis at ICU admission was the only significant predictor of ESZs or PEDs (odds ratio 4.6, 95% confidence interval 1.9-12.7, p = 0.002). After controlling for age, coma, and organ dysfunction, the presence of ESZs or PEDs was associated with death or severe disability at hospital discharge (89% with ESZs or PEDs, vs. 39% if not; odds ratio 19.1, 95% confidence interval 6.3-74.6, p < 0.001). In this retrospective study of MICU patients monitored with cEEG, ESZs and PEDs were frequent, predominantly in patients with sepsis. Seizures were mainly nonconvulsive. Both seizures and periodic discharges were associated with poor outcome. Prospective studies are warranted to determine more precisely the frequency and clinical impact of nonconvulsive seizures and periodic discharges, particularly in septic patients.
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Posterior reversible encephalopathy syndrome (PRES) is characterized by vasogenic subcortical edema without infarction. It has been associated with hypertensive crises and with immunosuppressive medications but not with neuromyelitis optica (NMO). We reviewed the clinical and neuroimaging features of five NMO-immunoglobulin G (IgG) seropositive white women who experienced an episode of PRES and had a coexisting NMO spectrum disorder (NMOSD). We also tested for the aquaporin-4 (AQP4) water channel autoantibody (NMO-IgG) in 14 patients from an independently ascertained cohort of individuals with PRES. All five patients developed abrupt confusion and depressed consciousness consistent with PRES. The encephalopathy resolved completely within 7 days. Comorbid conditions or interventions recognized to be associated with PRES included orthostatic hypotension with supine hypertension, plasma exchange, IV immunoglobulin treatment, and high-dose IV methylprednisolone. Brain MRI studies revealed bilateral T2-weighted (T2W) hyperintense signal abnormalities, primarily in frontal, parieto-occipital, and cerebellar regions. Three patients had highly symmetric lesions and three had gadolinium-enhancing lesions. Follow-up neuroimaging revealed partial or complete disappearance of T2W hyperintensity or gadolinium-enhancing lesions in all five patients. Patients with PRES without NMOSD were uniformly NMO-IgG seronegative. Brain lesions in some patients with neuromyelitis optica spectrum disorder (NMOSD) may be accompanied by vasogenic edema and manifest as posterior reversible encephalopathy syndrome (PRES). Water flux impairment due to aquaporin-4 autoimmunity may predispose to PRES in patients with NMOSD who experience blood pressure fluctuations or who are treated with therapies that can cause rapid fluid shifts.
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Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological entity, characterized by typical neurological deficits, distinctive magnetic resonance imaging (MRI) features, and a usually benign clinical course. Although frequently seen in association with hypertensive conditions, many other predisposing factors, notably cytotoxic and immunosuppressant drugs have been associated with PRES. The aim of this study was to determine differences in the MR appearance of PRES according to various risk factors. Thirty consecutive patients with clinical and MRI findings consistent with PRES were included. We identified 24 patients with hypertension-related conditions, including 14 patients with preeclampsia-eclampsia, and six patients without hypertension, in whom PRES was associated with exposition to neurotoxic substances. Lesion distribution, extent of disease, and number of affected brain regions were compared between patients with PRES with and without hypertension, and patients with PRES with and without preeclampsia-eclampsia, respectively. No statistically significant differences in distribution of lesions and extent of disease were observed between patients with PRES with or without hypertension, and patients with or without preeclampsia-eclampsia, respectively. The number of affected brain regions was significantly higher in patients with preeclampsia-eclampsia (p = 0.046), and the basal ganglia region was more frequently involved in these patients (p = 0.066). Apart from a significant higher number of involved brain regions and a tendency for basal ganglia involvement in patients with PRES associated with preeclampsia-eclampsia, the MRI appearance of patients with PRES does not seem to be influenced by predisposing risk factors.
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Hypertensive encephalopathy is a syndrome consisting of headache, seizures, visual changes, and other neurologic disturbances in patients with elevated systemic blood pressure. The purpose of this study was to analyze the imaging findings in 14 patients with hypertensive encephalopathy. CT (n = 13), MR (n = 12), and single-photon emission computed tomography (n = 2) examinations performed in these patients before and after resolution of symptoms were reviewed. Eight had the preeclampsia-eclampsia syndrome, and six had hypertensive encephalopathy due to other causes. CT and MR findings in all patients having these examinations were indicative of edema in the cortex and subcortical white matter in the occipital lobes. Two of the 14 patients also had similar findings in the cerebellum and frontal lobes. Single-photon emission computed tomography showed increased vascular perfusion adjacent to areas that appeared abnormal on CT and MR. The findings on the imaging studies resolved on follow-up examinations performed after the hypertension was corrected. Our results suggest that the radiologic findings associated with hypertensive encephalopathy may be useful in establishing the diagnosis in the appropriate clinical setting.
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Three women presenting with toxemia of pregnancy revealed reversible increased signal intensity on T2-weighted images by MRI. Overall, neuroradiologic techniques, with MRI the most sensitive, help elucidate the pathophysiology and facilitate the diagnosis in this condition.
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A 24-year-old woman with eclamptic convulsions during her 25th week of gestation is presented. Magnetic resonance imaging revealed multiple hyperintense foci within the cerebral cortex, most likely related to cerebral ischemia and edema. The MR abnormalities resolved completely within 1 week.
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We describe a patient who experienced focal cerebral and brainstem ischemia in the setting of postpartum eclampsia. Cerebral angiography showed spasm of large- and medium-caliber arteries. This case provides rare documentation that vasospasm may account for cerebral ischemia in eclamptic women with focal signs. This observation suggests that in such patients cerebral angiography may be informative and useful.
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Cerebral vasospasm was demonstrated with MR angiography in a patient with preeclampsia. MR angiography 5 days after the onset of symptoms clearly demonstrated diffuse intracranial vasospasm. The follow-up study confirmed the resolution of vasospasm successfully.
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In some patients who are hospitalized for acute illness, we have noted a reversible syndrome of headache, altered mental functioning, seizures, and loss of vision associated with findings indicating predominantly posterior leukoencephalopathy on imaging studies. To elucidate this syndrome, we searched the log books listing computed tomographic (CT) and magnetic resonance imaging (MRI) studies performed at the New England Medical Center in Boston and Hôpital Sainte Anne in Paris; we found 15 such patients who were evaluated from 1988 through 1994. Of the 15 patients, 7 were receiving immunosuppressive therapy after transplantation or as treatment for aplastic anemia, 1 was receiving interferon for melanoma, 3 had eclampsia, and 4 had acute hypertensive encephalopathy associated with renal disease (2 with lupus nephritis, 1 with acute glomerulonephritis, and 1 with acetaminophen-induced hepatorenal failure). Altogether, 12 patients had abrupt increases in blood pressure, and 8 had some impairment of renal function. The clinical findings included headaches, vomiting, confusion, seizures, cortical blindness and other visual abnormalities, and motor signs. CT and MRI studies showed extensive bilateral white-matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres, but the changes often involved other cerebral areas, the brain stem, or the cerebellum. The patients were treated with antihypertensive medications, and immunosuppressive therapy was withdrawn or the dose was reduced. In all 15 patients, the neurologic deficits resolved within two weeks. Reversible, predominantly posterior leukoencephalopathy may develop in patients who have renal insufficiency or hypertension or who are immunosuppressed. The findings on neuroimaging are characteristic of subcortical edema without infarction.
Article
The purpose of this study is to describe the neuroimaging (CT and MR imaging) findings in liver transplant patients who develop severe neurologic side effects during immunosuppressive therapy with tacrolimus and to correlate these findings with clinical signs and tacrolimus levels in blood. Brain CT and/or MR imaging was performed on six patients who developed neurologic symptoms while receiving tacrolimus in the post-transplant period. All patients were evaluated by the neurology staff, and imaging studies were independently interpreted by three neuroradiologists. Trough tacrolimus levels in blood were measured with the IMX immunoassay and were correlated with neurologic symptoms and imaging findings. Imaging abnormalities were observed in five of six patients during the course of their neurologic illnesses. For each patient, neurologic symptoms began when the tacrolimus level in blood was at a peak, exceeding the therapeutic limit in all but one case. In five patients, neurologic symptoms eventually resolved after the tacrolimus dose was reduced or after the drug was stopped. Multifocal low attenuation of white matter was the predominant finding seen on CT images, and matching hyperintense white matter foci were observed on long-TR MR images. In three patients, clinical recovery was accompanied by reversal of the white matter abnormalities seen on CT and MR images. Immunosuppressive therapy with tacrolimus may produce neurologic side effects that are associated with brain CT and MR imaging abnormalities. Resolution of symptoms and reversal of imaging findings occur when the tacrolimus dose is reduced.
Article
Tacrolimus (FK506) has recently been approved for immunosuppression in organ transplantation, although its use is accompanied by a wide spectrum of neurotoxic side effects. We describe the clinical, radiological, and pathological features of 3 cases of tacrolimus-related leukoencephalopathy. The syndrome of immunosuppression-related leukoencephalopathy is proposed as an uncommon neurological syndrome occurring in patients with organ transplants involving demyelination, in particular in the parieto-occipital region and centrum semiovale. Although the syndrome is not associated with a particular (absolute) serum level of tacrolimus, it resolves spontaneously upon decreasing the dose. The tacrolimus-related syndrome has a similar radiographic and pathologic appearance as the analogous syndrome that occurs in patients taking cyclosporine.
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The pathophysiology of eclampsia remains unclear. While the majority of patients develop reversible T2 hyperintense signal abnormalities on MR scans and reversible neurological deficits, some patients do develop infarctions (permanent T2 hyperintense abnormalities) and permanent neurological impairment. Routine MRI cannot prospectively differentiate between these two patient groups. Echo-planar diffusion-weighted imaging, however, is a new technique that clearly differentiates between cytotoxic and vasogenic edema. A 30-year-old woman developed symptoms consistent with eclampsia 24 hours after delivering premature twins. An MRI demonstrated extensive, diffuse T2 hyperintense signal abnormalities involving subcortical white matter and adjacent gray matter with a posterior predominance, consistent with either infarction or hypertensive ischemic encephalopathy. Diffusion-weighted images demonstrated increased diffusion, consistent with vasogenic edema and hypertensive ischemic encephalopathy. Unlike routine MRI, diffusion-weighted imaging reliably differentiates between vasogenic edema and cytotoxic edema. Consequently, in eclamptic patients diffusion-weighted imaging can afford clear differentiation between hypertensive ischemic encephalopathy and infarction, two very different entities with very different treatment protocols. Diffusion-weighted imaging should be performed in all eclamptic patients and should greatly affect their management.
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Diffuse subcortical MRI signal abnormalities were seen during a subacute exacerbation in a patient with Hashimoto's encephalopathy. The patient had an excellent clinical response to corticosteroids. Clinical recovery paralleled normalization of MRI abnormalities and lowering of thyroid microsomal antibody titer.
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The etiology of preeclampsia is unknown. At present, 4 hypotheses are the subject of extensive investigation, as follows: (1) Placental ischemia-Increased trophoblast deportation, as a consequence of ischemia, may inflict endothelial cell dysfunction. (2) Very low-density lipoprotein versus toxicity-preventing activity-In compensation for increased energy demand during pregnancy, nonesterified fatty acids are mobilized. In women with low albumin concentrations, transporting extra nonesterified fatty acids from adipose tissues to the liver is likely to reduce albumin's antitoxic activity to a point at which very-low density lipoprotein toxicity is expressed. (3) Immune maladaptation-Interaction between decidual leukocytes and invading cytotrophoblast cells is essential for normal trophoblast invasion and development. Immune maladaptation may cause shallow invasion of spiral arteries by endovascular cytotrophoblast cells and endothelial cell dysfunction mediated by an increased decidual release of cytokines, proteolytic enzymes, and free radical species. (4) Genetic imprinting-Development of preeclampsia-eclampsia may be based on a single recessive gene or a dominant gene with incomplete penetrance. Penetrance may be dependent on fetal genotype. The possibility of genetic imprinting should be considered in future genetic investigations of preeclampsia.
Article
In eclampsia, MR imaging shows reversible T2 hyperintensities in a parietal and occipital distribution. Findings on diffusion-weighted images suggest that these abnormalities are areas of vasogenic edema. We describe the presence of both cytotoxic and vasogenic edema, as detected by diffusion-weighted imaging, in a woman with eclampsia. Follow-up MR imaging showed that the regions of cytotoxic edema progressed to cerebral infarction. This case suggests that diffusion-weighted imaging allows the early detection of ischemic infarcts in patients with eclampsia.
Article
We assessed the utility of fluid-attenuated inversion-recovery (FLAIR) and diffusion-weighted (DWI) images in investigation of tacrolimus (FK-506) encephalopathy, and to see whether we could predict its cause from clinical and imaging data. In seven patients with presumed FK-506 toxicity the areas involved on MRI were similar to those in cyclosporin A (CsA) toxicity. The abnormal signal was most evident on FLAIR in all cases. In three of four patients who underwent DWI, no diffusion abnormalities were detected; in the remaining patient, increased diffusion was seen in the deep white matter bilaterally on the apparent diffusion coefficient map, consistent with the findings on T2-weighted spin-echo and FLAIR images. Five of the six patients for whom we had clinical data showed sudden changes in electrolyte or fluid equilibrium due to diarrhoea, a polyuria or oliguria one day before or on the day of onset of the central nervous system disturbances. We speculate that FK-506 encephalopathy is triggered by the disturbance of the electrolyte and/or fluid equilibrium, given a certain serum level of FK-506.
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Severe sepsis, defined as sepsis with acute organ dysfunction, is associated with high morbidity and mortality rates. The development of novel therapies for sepsis is critically dependent on an understanding of the basic mechanisms of the disease. The pathophysiology of severe sepsis involves a highly complex, integrated response that includes the activation of a number of cell types, inflammatory mediators, and the hemostatic system. Central to this process is an alteration of endothelial cell function. The goals of this article are to (1) provide an overview of sepsis and its complications, (2) discuss the role of the endothelium in orchestrating the host response in sepsis, and (3) emphasize the potential value of the endothelium as a target for sepsis therapy.
Article
Posterior reversible encephalopathy syndrome (PRES) is a rare, recently described neurologic condition identifiable by clinical presentation and magnetic resonance image (MRI) appearance. It is associated with renal insufficiency, hypertension, and rheumatologic diseases. Patients present with headache, seizures, loss of vision and altered mental function, and a pattern on imaging studies of predominantly transient, posterior cerebral hyperintensities on T2-weighted MRI. There is a high likelihood of presentation of this syndrome to a rheumatologist. Three recent cases of systemic lupus erythematosus (SLE) with PRES, along with 9 previously reported cases, are reviewed. All 3 patients presented with seizures and subacute visual changes in association with lupus nephritis. The first presented with hypertension, complete visual field loss, and status epilepticus 2 weeks after starting oral cyclosporine therapy for refractory lupus nephritis. The second patient was normotensive and presented with seizures and visual symptoms while in hospital with SLE-related pancreatitis and nephritis. The third patient had headache and seizures with severe lupus disease activity including nephritis, pancytopenia, and pulmonary hemorrhage. Cranial MRI showed predominantly posterior signal abnormalities on T2-weighted images, which resolved after cessation of cyclosporine in the first case, treatment with IV cyclophosphamide in the second case, and treatment with cyclophosphamide and plasmapheresis in the final case. Literature review showed that PRES is a manifestation of SLE or a consequence of therapy with calcineurin inhibitors or rituximab. The hallmark features are visual loss and seizures. Severe hypertension (> 170/110 mm Hg) and renal failure were present in the majority of previously identified cases of SLE and PRES. Our second case was normotensive but had marked lupus disease activity. PRES can lead to cerebral infarction. With increasing availability of MRI, PRES will be identified more frequently. Swift action to identify potential offending agents, controlling hypertension, and treating active disease can lead to reversal of radiologic and neurologic findings.
Article
Although the term posterior reversible encephalopathy syndrome (PRES) was popularized because of the typical presence of vasogenic edema in the parietal and occipital lobes, other regions of the brain are also frequently affected. We evaluated lesion distribution with CT and MR in a large cohort of patients who experienced PRES to comprehensively assess the imaging patterns identified. The locations of the PRES lesion at toxicity were comprehensively identified and tabulated in 136 patients by CT (22 patients) and MR (114 patients) imaging including the hemispheric, basal ganglial, and infratentorial locations. Clinical associations along with presentation at toxicity including blood pressure were assessed. Vasogenic edema was consistently present in the parietal or occipital regions (98%), but other locations were common including the frontal lobes (68%), inferior temporal lobes (40%), and cerebellar hemispheres (30%). Involvement of the basal ganglia (14%), brain stem (13%), and deep white matter (18%) including the splenium (10%) was not rare. Three major patterns of PRES were noted: the holohemispheric watershed (23%), superior frontal sulcal (27%), and dominant parietal-occipital (22%), with additional common partial or asymmetric expression of these primary PRES patterns (28%). Involvement of the frontal lobe, temporal lobe, and cerebellar hemispheres is common in PRES, along with the occasional presence of lesions in the brain stem, basal ganglia, deep white matter, and splenium. Three primary PRES patterns are noted in the cerebral hemispheres, along with frequent partial or asymmetric expression of these PRES patterns. Awareness of these patterns and variations is important to recognize PRES neurotoxicity more accurately when present.
Article
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. To retrospectively identify patients with RPLS with a characteristic clinical presentation and neuroimaging abnormalities and documented improvement on repeated neuroimaging. Retrospective. Mayo Clinic. Thirty-six patients with RPLS. 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. 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. 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.
Article
A 40-year-old woman was admitted to our hospital with disturbance of consciousness and seizure. We diagnosed encephalopathy associated with autoimmune thyroid disease (EAATD). Fluid-attenuated inversion recovery and diffusion-weighted MRI demonstrated hyperintense lesions in the left occipitotemporal lobe on admission, but these findings disappeared on day 11 without neurological deficits, compatible with posterior reversible encephalopathy syndrome (PRES). We report here this case of autoimmune thyroid disease presenting as PRES.
A reversible posterior leukoencephalopathy syndrome A. Diffusion-weighted imaging shows cytotoxic and vasogenic edema in eclampsia
  • Rb Schwartz
  • S Koch
  • A Rabinstein
  • S Falcone
Schwartz RB. A reversible posterior leukoencephalopathy syndrome [letter]. N Engl J Med. 1996;334(26):1743. 25. Koch S, Rabinstein A, Falcone S, et al. A. Diffusion-weighted imaging shows cytotoxic and vasogenic edema in eclampsia. AJNR Am J Neuroradiol. 2001;22(6):1068-1070.