Posterior reversible encephalopathy syndrome

Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological entity with typical symptoms and symmetric high-signal intensity lesions in the bilateral parieto-occipital lobes on T2-weighted or fluid-attenuated inversion recovery (FLAIR) MRI. We described three patients with PRES of varied etiologies. Patient 1 was a young man with severe hypertension who presented with headache and visual disturbance. Patient 2 had leukemia and was receiving umbilical cord blood cell transplantation with immunosuppressant, and developed PRES with convulsions. Patient 3 was a pregnant woman with renal failure, who repeatedly developed PRES with convulsions. FLAIR and apparent diffusion coefficient mapping were useful in detecting PRES lesions in our patients, although diffusion-weighted imaging and CT scans had limited use in the diagnosis. Adequate and prompt treatment with antihypertensive medication immediately ameliorated the symptoms, with improvement of abnormal MRI findings. In previous reports, delayed diagnosis might have affected the prognosis. Further work on the clinical manifestations of PRES and its therapy is required.     

Clinical predictors and differential diagnosis of posterior reversible encephalopathy syndrome

The aim of our study is to determine the clinical predictors and the differential diagnosis of posterior reversible encephalopathy syndrome (PRES) in patients presenting with acute neurological symptoms and risk factors for PRES. Using the diagnostic algorithm for PRES from Fugate and Rabinstein (Lancet Neurol 14(9):914–925, 1), we carried out a retrospective study on 220 patients, presenting with acute neurological symptoms such as seizures, encephalopathy, headache, visual disturbances or other focal neurological signs that appear in the clinical setting of risk factors such as hypertension/blood pressure fluctuations, chemotherapy, renal failure, autoimmune disorders, or eclampsia, in whom imaging of the brain was performed to exclude PRES. Seventeen percent of patients had a radiologically confirmed diagnosis of PRES. Univariable logistic regression showed a significant association between PRES and epileptic seizures, encephalopathy, hypertension, chemotherapy and renal failure. Multivariable logistic regression of acute neurological symptoms and risk factors showed a significant association of epileptic seizures, encephalopathy, visual disturbances, hypertension and chemotherapy with PRES. Using these variables to predict PRES yielded a discriminative ability (AUC) equal to 0.793. Diagnoses when PRES was not confirmed included primary or secondary headaches (26%), toxic-metabolic encephalopathy (21%), vascular pathology (12%) and other less frequent disorders. Epileptic seizures, encephalopathy, visual disturbances, hypertension, renal failure and chemotherapy were the best clinical predictors of PRES, while headache, immune suppression and autoimmune disease were not useful for the clinical diagnosis of PRES in our study.     

Posterior reversible encephalopathy and Guillain-Barré syndrome in a single patient: Coincidence or causative relation?

We report 62-year-old female patient with coincident posterior reversible encephalopathy syndrome (PRES) and Guillain-Barré syndrome (GBS). The first presentation of PRES was a generalised tonic-clonic seizure. A risk factor for PRES was acute arterial hypertension. The diagnosis of PRES was established by MRI (magnetic resonance imaging) and hypertension was treated with labetalol 800mg daily followed by regression of symptoms of PRES. Two days after the seizure the first motor signs of GBS presented with a weakness in both upper arms. The diagnosis of GBS was finally established 6 days after the seizure by clinical evolution, lumbar puncture and electrophysiological findings. After treatment of GBS with intravenous immunoglobulins (IVIg), antihypertensive therapy could be phased out and finally stopped. The patient was discharged after 25 days without any medication. At that time she was completely recovered from PRES and recovering well from GBS. The acute arterial hypertension, the provoking factor of PRES, was probably caused by an autonomic dysfunction in the context of GBS before motor signs of GBS were present but we speculate also that there are other GBS related factors playing a role in PRES. This hypothesis is based on the relatively high coincidence of these two rare syndromes which appears from a review of the literature. One other possible mechanism can be the influence of cytokines, produced in the context of a GBS, on the permeability of blood brain barrier.     

Acute glomerulonephritis presenting with PRES: a report of 4 cases

OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) occurs most commonly in the setting of known hypertension or use of immunosuppressive agents. DESIGN AND METHODS: We report four previously-well children who presented acutely with altered mentation, seizures and visual disturbances and were diagnosed with PRES. RESULTS: Only one child had a history of gross hematuria prior to the seizure. All four were discovered to be hypertensive only after onset of their neurological symptoms, and were subsequently diagnosed with glomerulonephritis. All four had rapid resolution of neurological symptoms with adequate treatment of hypertension. CONCLUSIONS: Blood pressure must be measured promptly in all children presenting with these symptoms. If elevated, the diagnosis of PRES should be strongly considered and a workup for renal disease pursued.     

Seizure outcomes of posterior reversible encephalopathy syndrome and correlations with electroencephalographic changes

RationaleSeizures are among the most common clinical presentations of posterior reversible encephalopathy syndrome (PRES). This syndrome has rarely been reported to cause chronic epilepsy or persistent cortical dysfunction. The prognostic value of EEG findings during PRES is unknown. We retrospectively evaluated EEG characteristics in patients with PRES in a single medical center. We also evaluated the long-term outcome regarding seizure occurrence beyond the acute phase in these patients.MethodsWe searched a radiology database at the University of Minnesota from 1997 to 2012 to identify patients with clinically and radiologically diagnosed PRES. Among the patients with PRES, we reviewed MRI images, EEG findings, clinical manifestations including seizure occurrences, and clinical outcomes beyond the acute phase.ResultsSeventy-five patients were included in the study. Fifty-eight out of seventy-five (77.3%) patients with PRES had seizures. A total of 48 EEG studies were performed in 38 patients. Generalized slowing was the most common EEG pattern. Among the 38 patients who had EEGs, 37 (97.3%) patients had diffuse or focal slowing of the background, and 11 (28.9%) patients had IEDs. Four out of seventy-five (5.3%) patients had seizures later than one month beyond their hospitalization for PRES. None of these 4 patients had seizures before the episode of PRES. Two patients developed chronic epilepsy, with seizures occurring later than one year after the PRES.ConclusionMost patients who had seizures or who had epileptiform activities in EEG during PRES did not subsequently develop chronic epilepsy. No patient developed chronic epilepsy in the absence of clinical seizures during PRES. Posterior reversible encephalopathy syndrome may infrequently be associated with subsequent development of symptomatic epilepsy.     

Is hypertension predictive of clinical recurrence in posterior reversible encephalopathy syndrome?

Posterior reversible encephalopathy syndrome (PRES) has a distinctive clinical presentation and typical neuroimaging findings. However, data on its clinical course and recurrence are scarce. This study aims to investigate its clinical profile and factors that predict recurrence. We included patients diagnosed with PRES between 2005 and 2010 and collected data on demographics, presenting symptoms, co-morbidities, risk factors, clinical parameters, MRI findings, complications and recurrence. Patients were categorized into two groups: PRES due to primary hypertension and PRES due to secondary causes. Correlation with presenting symptoms, radiological features, and recurrence were analyzed. PRES was identified in 28 patients. Fourteen (50%) had primary hypertension. Secondary causes included immunosuppression-related (39%), preeclampsia/eclampsia (7%), and marijuana-intake-related (4%) causes. Patients presented with altered mental status (79%), headache (75%), seizure (68%), visual disturbance (39%) and hemiparesis (21%). On MRI 93% had the typical parietal–occipital involvement. The frontal lobe was affected in 64%, cerebellum in 29%, brainstem in 21%, and basal ganglia in 11%. About 36% had cortical involvement; 21% had diffusion-restricted lesions. Non-aneurysmal subarachnoid haemorrhage was found in 18% of patients and intracerebral hemorrhage in 14% of patients. No significant difference existed in presenting symptoms and the MRI distribution of vasogenic edema between the primary hypertension group and the secondary causes group. Recurrence occurred in four patients (14.3%, 95% confidence interval 4.2–33.7) and was significantly associated (p = 0.05) with primary hypertension as the etiology. Intensive monitoring and treatment of hypertension is recommended for reducing morbidity.     

Spontaneous posterior fossa hemorrhage associated with low-molecular weight heparin in an adolescent recently diagnosed with posterior reversible encephalopathy syndrome: case report and review of the literature

Background Posterior reversible encephalopathy syndrome (PRES) constitutes a well-described clinical entity, classically entailing characteristic edematous lesions on imaging studies in a patient with altered mental status, visual disturbances, headache, and seizures. PRES is reversible in the vast majority of cases, although progression to ischemia and hemorrhage has been documented.Methods We report a case of a 16-year-old male with chronic renal failure who developed PRES during a hypertensive crisis. The hypertension was successfully managed and PRES-associated symptomatology showed complete regression. However, approximately 2 months later, the patient returned with a spontaneous posterior fossa hemorrhage. Two weeks before this second admission, treatment with low-molecular weight heparin had been initiated. The patient finally succumbed, despite surgical evacuation. The pertinent literature is reviewed.Conclusion We suggest that the administration of low-molecular weight heparin in the setting of recent PRES might increase the risk for the development of intracranial hemorrhage.     

Isolated brainstem involvement in posterior reversible encephalopathy syndrome: a case report and review of the literature

Posterior reversible encephalopathy syndrome (PRES) is a clinical–radiological syndrome, reversible vasogenic edema predominantly within parieto-occipital regions. However, isolated brainstem involvement in PRES has been rarely reported, little is known about its clinical manifestations, radiological features and outcomes. We reported a case with PRES with only brainstem involvement and performed a systematic review of published cases. Twenty-four cases, together with our case, were included in the analysis. Mean age was 43?years and 63% were males. Hypertension (50%), nephropathy (25%) and chemotherapy (21%) were the major risk factors. All patients except two had acute hypertension and 50% of patients had renal dysfunction at onset. The most common symptoms were altered consciousness (46%) and headache (46%), Seizure was only presented in 21% of patients. All patients except two were treated with antihypertension. Most patients recovered to their neurological baseline. Clinicians should recognize this unique variant finding in PRES. which always affects males with severe hypertension, especially combined with renal dysfunction. Antihypertensive treatment is the most widely used therapy. Outcome is usually well.     

Posterior reversible encephalopathy syndrome: a case of unusual diffusion-weighted MR images

Posterior reversible encephalopathy (PRES) represents an uncommon entity related to multiple pathologies, the most common of which is hypertensive crisis. PRES is classically characterized as symmetrical parieto-occipital edema, but may affect other areas of the brain. Diffusion-weighted magnetic resonance imaging (DWI) is important for differentiating between vasogenic and cytotoxic edema. We present here the case of a 43-year-old woman, known to suffer from arterial hypertension and severe renal failure, who developed PRES with restricted apparent diffusion coefficients (ADC) in various cerebral areas, suggesting irreversible tissue damage. Nevertheless, follow-up cranial MRI revealed complete remission, indicating that restricted diffusion does not always lead to cell death in this pathology. The underlying pathophysiological mechanism is not well understood. Such reversibility of diffusion anomalies has already been reported with transient ischemia, vasospasm after subarachnoid hemorrhage and epilepsy but, to our knowledge, never before in PRES.     

Posteriores reversibles Enzephalopathiesyndrom

Posterior reversible encephalopathy syndrome (PRES) is a clinically and radiologically defined syndrome characterized by seizures, headaches, visual disturbances, and altered mental status or focal neurological deficits. Imaging frequently shows abnormalities in the posterior brain regions, especially the occipital and the parietal lobes. PRES has been described in association with a wide spectrum of underlying causes. The most common conditions include hypertension, renal disease, preeclampsia/eclampsia, or status post transplantation. The pathophysiology of PRES has not been sufficiently elucidated as yet; however, endothelial damage caused by different mechanisms possibly plays a central role. The prognosis of PRES is usually benign when intensive care and withdrawal of the causative agent is instituted early. Delay in diagnosis and treatment may lead to complications and permanent neurological deficits. The clinical and radiological landmarks of this syndrome as well as causes, pathophysiological hypotheses, and the basic therapeutic principles of PRES are the subject of the present review.     

The determinants of neurological phenotypes during acute hypertensive crises – a preliminary study

ABSTRACT

Background and Purpose: Acute blood pressure elevations lead to wide spectrum of neurologic manifestations, ranging from no overt neurologic symptoms to catastrophic events like ICH. Little is known regarding the determinants of this clinical variability. We determined clinical and imaging features of hypertensive crisis patients with normal neurological examination, ICH and posterior reversible encephalopathy syndrome (PRES).

Methods: Cranial MRI was performed in patients with hypertensive urgency or emergency but normal neurological examination. Their clinical characteristics, and imaging features regarding cerebral small vessel disease were compared to ICH and PRES patients.

Results: Hypertensive ICH patients (n = 58) were older, less likely to have hyperlipidemia, less commonly used calcium channel blockers, and had higher burden of chronic cSVD features in comparison to hypertensive crisis patients with normal neurological findings (n = 51). Multivariate analyses revealed cSVD burden score (p = 0.003) to be related with ICH, while higher admission blood pressure levels (p < 0.001), hyperlipidemia (p = 0.006) and calcium channel blocker usage (p = 0.005) were more common in patients with normal neurological examination. The PRES (n = 9) group was comprised of younger patients with recent history of hypertension and low burden of cSVD.

Conclusions: Hypertensive surge is associated with ICH when cSVD burden is high, probably caused by microvascular dysfunction secondary to long-standing hypertension, while the episode causes no structural damage if this burden is less. Although our observations are exploratory, short term but severe hypertension manifests with PRES possibly due to the absence of adaptive changes.     

Unusual presentation of Takayasu's arteritis as posterior reversible encephalopathy syndrome

Takayasu''s arteritis is a chronic, idiopathic, inflammatory disease primarily affecting aorta and its branches. It mainly affects young females in the age group of 10-30 years. Various atypical presentations of Takayasu''s arteritis have been described in children. These atypical presentations can cause delayed diagnosis resulting in increased morbidity and mortality. Posterior reversible encephalopathy syndrome (PRES) is a neuroradiologic condition associated with headache, seizures, altered sensorium, visual disturbances, and characteristic lesions on neuroimaging. We report a child with Takayasu''s arteritis who presented a posterior reversible encephalopathy syndrome. He also had associated abdominal tuberculosis for which anti-tuberculous treatment was started. PRES was diagnosed by magnetic resonance imaging with fluid-attenuated inversion recovery sequences. The child was started on nifedipine and propranolol. The child regained his consciousness within 48 h of admission. Prompt treatment of hypertension led to rapid reversal of neurological symptoms. In view of hypertension a computed tomography aortogram was done, which showed features suggestive of high grade (>75%) focal proximal left renal artery stenosis. EULAR (European League Against Rheumatism)/PReS (Paediatric Rheumatology European Society) consensus criteria was used for the diagnosis of Takayasu''s arteritis in our patient. Percutaneous transluminal balloon angioplasty of the stenotic left renal artery was performed. Post-angioplasty, nifedipine was gradually omitted and oral propranolol was continued.     

Partial status epilepticus related to independent occipital foci in posterior reversible encephalopathy syndrome (PRES)

Posterior reversible encephalopathy syndrome (PRES) is an acute disorder characterised by a variable association of neurologic symptoms with potentially reversible oedematous abnormalities mainly in the parieto-occipital regions of the brain. Despite the significant incidence of seizures, the EEG characteristics of epileptic disorders related to PRES have rarely been investigated. We report the case of an 85-year-old man who presented with generalised tonic-clonic seizures and prolonged disturbances of consciousness as clinical manifestations of PRES due to moderate exacerbation of chronic hypertension. An EEG performed during an alteration of mental function displayed a pattern of partial status epilepticus (SE) in both temporo-parieto-occipital regions. The seizure activity originated from two independent epileptic foci located in the occipital area of each hemisphere and could be related to the parenchymal abnormalities of PRES. The EEG pattern of partial SE related to independent occipital foci illustrates a distinctive seizure disorder that could be characteristic of PRES in adult patients.

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Sommario  La sindrome della encefalopatia posteriore reversibile (PRES) è caratterizzata da una associazione di sintomi neurologici acuti (principalmente cefalea, vomito, alterazione della coscienza, crisi epilettiche e cecità corticale) e lesioni edematose potenzialmente reversibili delle regioni cerebrali parieto-occipitali. Descriviamo il caso di un soggetto maschile di 85 anni che ha manifestato crisi tonico-cloniche generalizzate ed un prolungato disturbo della coscienza come manifestazione di PRES secondaria a moderato incremento della pressione arteriosa. L’EEG ha evidenziato uno stato di male epilettico parziale legato a localizzazioni epilettogene occipitali bilaterali ed indipendenti. L’attività epilettica era correlabile alle alterazioni parenchimali della PRES. Il quadro EEGrafico osservato configura un disturbo epilettico raramente descritto nella letteratura, che potrebbe essere caratteristico della PRES in pazienti adulti.

     

Posterior reversible encephalopathy syndrome in children: report of three cases

Purpose

Posterior reversible encephalopathy syndrome (PRES) is a condition characterized by varying degrees of headache, nausea, vomiting, visual disturbances, focal neurologic deficit, and seizures due to severe systemic hypertension. The knowledge of secondary hypertension in children is most commonly due to renal abnormalities, suggesting that the leading cause of PRES in childhood is renal diseases.

Methods

Three pediatric patients who developed PRES due to various underlying renal diseases were reviewed.

Results

The etiology of hypertension of our patients was all renal problems including atrophic kidney, hydronephrosis secondary to reflux nephropathia, nephrotic syndrome, and acute poststreptococcal glomerulonephritis. While two of them had typical of the parieto-occipital and frontoparietal involvement, the other had brain stem involvement. All of the patients were recovered by the control of high blood pressure.

Conclusion

Primary involvement of the brain stem is rare in children. PRES should be taken into account, especially in children with renal disease in the appropriate clinical settings.     

Posterior reversible encephalopathy syndrome following hemodynamic treatment of aneurysmal subarachnoid hemorrhage-induced vasospasm

Posterior reversible encephalopathy syndrome (PRES) is an uncommon but significant complication of hemodynamic therapy after aneurysmal subarachnoid hemorrhage (aSAH)-induced vasospasm. We performed a PubMed literature search for the period January 1999 to January 2015 using the search terms “posterior reversible encephalopathy syndrome”, “subarachnoid hemorrhage”, “vasospasm”, and “hypertensive encephalopathy”, and identified nine cases of PRES after aSAH-induced vasospasm in the literature. We also present a 63-year-old man with aSAH complicated by vasospasm treated with hemodynamic augmentation who subsequently developed PRES. Imaging following development of PRES symptoms shows vasogenic edema in the white matter of the parietal and occipital lobes. Age, sex, history of hypertension, and baseline blood pressure were variable among patients in the literature review. In all cases, patients improved both from a radiological and clinical perspective following blood pressure reduction. To summarize, PRES is a rare complication of hemodynamic therapy for vasospasm following aSAH. The literature at the time of writing demonstrates no common pattern with regard to patient demographics, medical history, or mode of treatment for symptomatic vasospasm. Given its sporadic and unpredictable nature, considering PRES in the differential diagnosis is important when addressing neurological decline following hemodynamic treatment of vasospasm related to aSAH.     

Posterior Reversible Encephalopathy Syndrome Following a Scorpion Sting

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic entity not yet understood, that is present with transient neurologic symptoms and particular radiological findings. The most common imaging pattern in PRES is the presence of edema in the white matter of the posterior portions of both cerebral hemispheres. The cause of PRES is unclear. We report a case of 13‐year‐old male who was stung by a scorpion and developed a severe headche, visual disturbance, and seizures and had the diagnosis of PRES with a good outcome. Numerous factors can trigger this syndrome, most commonly: acute elevation of blood pressure, abnormal renal function, and immunosuppressive therapy. There are many cases described showing the relationship between PRES and eclampsia, transplantation, neoplasia and chemotherapy treatment, systemic infections, renal disease acute, or chronic. However, this is the first case of PRES following a scorpion sting.     

Posterior reversible encephalopathy syndrome as a complication of acute lupus activity

Objectives

We aimed to describe the clinical and imaging characteristics; associated risk factors and neurological outcome of posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE).

Methods

From October 2001 to January 2007, we identified patients with SLE and the criteria for PRES in our institution, which is a tertiary-care referral center for patients with SLE; the patients were evaluated at baseline and followed to determine the clinical outcome.

Results

We identified 22 episodes of PRES in 21 patients; 20 (95.2%) were women, mean age of onset was 24.9 ± 8.6 years, all patients had high systemic activity (SLEDAI scores from 12 to 39). Acute hypertension was observed in 18 episodes (81.8%), and renal failure in 16 (72.7%); only 3 patients were on cyclophosphamide at the time of the onset of PRES. Persistent neurological deficit was observed in 2 cases; one patient died during the acute episode.

Conclusions

PRES is a central nervous system syndrome that is observed in SLE patients. It was associated mainly to high systemic activity, acute hypertension, and renal failure. Although reversibility is common, residual neurological damage may be observed.     

Posterior reversible encephalopathy syndrome in IgA vasculitis: Neuroimaging of a 14-year-old child

IgA vasculitis (IgAV) is a leukocytoclastic vasculitis and characterized by involvement of small vessels in skin, gastrointestinal system, joints, kidneys, and less frequently other organs. It is the commonest vasculitis in childhood and etiology is not completely known. Neurological manifestations of IgAV are very rare and usually seen in patients with severe hypertension or as an uncommon feature such as peripheral neuropathy. Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiologic entity characterized with temporary vasogenic edema developing typically in posterior circulation of the brain and has been reported as a rare manifestation of IgAV. In this paper, a PRES case of 14-year-old male with IgAV is reported and etiopathogenesis was discussed with literature. Diagnosis was made by magnetic resonance imaging because of the existence of neurological symptoms (headache and visual loss) during the course of disease. His radiological findings have resolved with therapy. Although neurological involvement is a rare manifestation in IgAV, we recommend magnetic resonance imaging in such patients for diagnosis and evaluation of complications.     

The Clinical and Radiological Spectrum of Posterior Reversible Encephalopathy Syndrome: A Retrospective Series of 24 Patients

ABSTRACT

BACKGROUND AND PURPOSE

Some patients with posterior reversible encephalopathy syndrome (PRES) present with atypical clinical and neuroimaging findings. The purpose of this study is to review the clinical and neuroimaging findings in patients diagnosed with PRES.

METHODS

We retrospectively included all patients diagnosed with PRES between January 2005 and March 2009. We reviewed demographic, clinical and laboratory data, neuroimaging findings, and prognosis.

RESULTS

Twenty‐four patients were included with a median age of 19 years. The clinical presentations included seizures (91.7%), headache (83.3%), visual disturbance (62.5%), encephalophathy (29.2%), and paralysis (8.3%). Co‐morbidities included systemic lupus erythematous (29.2%), kidney disease (20.8%), eclampsia (20.8%), renal artery stenosis (12.5%), Takayasu arteritis (4.2%), Sheehan's syndrome (4.2%), allergic purpura (4.2%), and acute intermittent porphyria (4.2%). Acute elevation of blood pressure was found in 22 patients (91.7%). Ten patients (41.7%) used steroids or immunosuppressants, three (12.5%) had acute renal failure before the symptoms. Atypical neuroimaging features included involvement of the frontal lobe (54.2%), basal ganglia (4.2%) or cortex (8.3%), and irreversible lesions (3/18, 16.7%).

CONCLUSIONS

A large proportion of patients with PRES may present with atypical neuroimaging findings. Apart from the known risk factors, such as hypertension, immunosuppressants, and renal failure of various etiologies may be the precipitants of PRES.

     

A case of catatonia due to posterior reversible encephalopathy syndrome treated successfully with antihypertensives and adjunctive olanzapine

Catatonia is a distinct neuropsychiatric syndrome with prominent motor manifestations. Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic syndrome usually precipitated by malignant hypertension. Given the overlapping neuropathology in both syndromes, we present a case of catatonia precipitated by PRES, with full resolution of the former after successful treatment of the latter.