You are here

Article Text

Article menu

Download PDFPDF

Review
Posterior reversible encephalopathy syndrome (PRES): diagnosis and management
Free
  1. James D Triplett1,
  2. Mansur Amirovich Kutlubaev2,
  3. Allan G Kermode3,4,
  4. Todd Hardy1,5
  1. 1 Neurology, Concord Hospital, Concord, New South Wales, Australia
  2. 2 Bashkir State Medical University, Ufa, Russian Federation
  3. 3 Perron Institute, University of Western Australia, Perth, Western Australia, Australia
  4. 4 CMMIT, Murdoch University, Murdoch, Western Australia, Australia
  5. 5 Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia
  1. Correspondence to Dr James D Triplett, Neurology, Concord Hospital, Concord, NSW 2139, Australia; James.triplett{at}health.nsw.gov.au

Abstract

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.

  • stroke

https://doi.org/10.1136/practneurol-2021-003194

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    Introduction

    Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological diagnosis that is based on a combination of typical clinical features and risk factors, and supported by magnetic resonance (MR) brain scan findings. Neurological symptoms can be multiple or occur in isolation and may evolve over the course of the acute phase of the disease. Its classical presentation is a combination of visual loss, headache, altered mental function, seizures and nausea, but may include other focal deficits including weakness, sensory disturbance or speech disturbance. The syndrome of PRES has many underlying causes and may result from medical treatments (eg, antineoplastic therapy) or may develop as part of a PRES-associated medical condition (eg, autoimmune disorders or eclampsia) (box 1).1 2

    Box 1

    Conditions associated with the development of PRES

    General conditions

    • Hypertension

    • Sepsis

    • Solid organ transplantation

    • Eclampsia and pre-eclampsia

    • Renal failure

    • Malignancy (solid organ and haematological)

    • Bone marrow transplantation

    • Stem cell transplantation

    • Hypomagnesaemia

    • Hypercalcaemia

    • Hypercholesterolaemia

    • Late radiation-associated encephalopathy, for example, SMART

    Autoimmune disorders

    • Rheumatoid arthritis

    • Crohn’s disease

    • Systemic lupus erythematosus

    • Scleroderma

    • Vasculitis

    • Neuromyelitis spectrum disorder

    Toxins

    • Scorpion poison

    • LSD intoxication

    • Ephedra overdose

    • Alcohol intoxication

    • Cocaine

    Cytotoxic and immunosuppressive medications

    • Hydroxydaunorubicin/adriamycin

    • Vinblastine/vincristine

    • Gemcitabine

    • Platinum-containing drugs: cisplatin, oxaliplatin and carboplatin

    • Bortezomib

    • Cyclophosphamide

    • Daunorubicin

    • Interferon therapy

    • Capecitabine, 5-fluorouracil

    • Cytarabine

    • Etoposide

    • Corticosteroids

    • Rituximab

    • Ciclosporin

    • Tacrolimus

    • Sirolimus

    • Mycophenolate mofetil

    • Methotrexate

    • Azathioprine

    Other medications

    • Lithium

    • Linezolid

    • Intravenous contrast

    • Intravenous immunoglobulin

    • Tyrosine kinase inhibitors

    PRES may develop at any age from infants to the elderly, but most frequently affects young or middle-aged adults, with a mean age of 45 years.1 3 There appears to be a female predominance, even after excluding patients with eclampsia.2 4 5 Among adults, PRES is present in up to 98% of patients with eclampsia6; in 2.7%–25% of patients following bone marrow transplantation7 8; in 0.4%–6% of patients following solid organ transplantation9; and less commonly (0.4%–0.8%) in end-stage renal disease or systemic lupus erythematosus.10–12

    PRES is in the differential diagnosis of many acute neurological presentations; the key to its diagnosis is to have a high index of suspicion in several scenarios when there are consistent findings on MR brain scan. The following sections deal with a clinical approach to the diagnosis and management of PRES.

    Acute evaluation

    Early on, what information do I need and what should I look for?

    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.1–3 Seizures affecting up to 74%–87% of patients1 2 and typically occur within 24–48 hours of presentation13 14; in a minority (3%–17%), seizures may evolve into status epilepticus, and rarely this is the presenting symptom.

    Headaches develop in up to half of patients with PRES3 and are usually dull, diffuse and gradual in onset. A thunderclap headache can occur in the context of PRES but should raise the suspicion of reversible cerebral vasoconstriction syndrome (RCVS). This condition occurs in the context of similar risk factors to PRES but is distinguished mainly by thunderclap headache, cerebral vasculopathy, infarction, and subarachnoid or intracranial haemorrhage.15–21

    Despite PRES often affecting the occipital regions, only 39% of patients have visual symptoms.1 3 These include reduced visual acuity, diplopia, visual field deficits, cortical blindness, colour vision abnormality and visual hallucinations. Focal neurological deficits such as aphasia and hemiparesis occur in 19% of patients.3

    Because the symptoms linked to PRES commonly occur in other conditions, its diagnosis is challenging and requires clear communication among clinicians and with radiologists. It is essential to review the patient’s current and medical history, medication charts and examination findings, including blood pressure and level of consciousness. Despite PRES being associated with accelerated hypertension, about 20%–30% of patients are normotensive or hypotensive during initial evaluation, especially when PRES is due to medications or systemic conditions other than hypertension or eclampsia.4 21

    PRES is associated with numerous conditions and iatrogenic causes (box 1), though most frequently with hypertension, renal failure, eclampsia, transplant and immunosuppressive use, sepsis, autoimmune disorders and exposure to cytotoxic medications.22–24 Rarer iatrogenic causes include linezolid,25 intravenous immunoglobulin26 and intoxication with LSD,27 cocaine,28 and even scorpion poisoning.29

    What baseline tests should I perform?

    MR scan of the brain assists with confirming the diagnosis of PRES and in showing its extent. It is therefore preferred to CT scanning due to its superior resolution, especially of posterior fossa structures. Nevertheless, CT scanning is usually the first form of imaging acutely and PRES is often diagnosed using CT scan alone. The typical CT and MRI features of PRES include almost symmetrical hemispheric vasogenic oedema affecting subcortical white matter and often extending to the overlying cortex, best seen on MRI with fluid-attenuation inversion recovery (FLAIR) sequences.22 MRI diffusion-weighted imaging usually confirms vasogenic oedema with absence of restricted diffusion. Postcontrast enhancement occurs in 38%–50% of patients23 30 31 in one of three patterns: a leptomeningeal pattern, a cortical pattern within regions of altered FLAIR signal or a combined pattern (figure 1).4 31

    Figure 1
    Figure 1

    Imaging findings in typical PRES. MR scan of the brain of a 39-year-old woman with PRES who presented with visual disturbance, seizure and fever. (A–C) Prominent cortical and subcortical white matter signal hyperintensity on T2 FLAIR involving bilateral occipital and left anterior parietal lobe, left frontal lobe, and the splenium of the corpus callosum with additional foci involving both the anterior left thalamus and posterior left putamen. There are also patchy hyperintense T2 FLAIR signal foci within the right cerebellar hemisphere. (D,E) Areas of diffusion-weighted imaging hyperintensity and hyperintense apparent diffusion coefficient signal involving the occipital cortex bilaterally consistent with vasogenic oedema, which corresponds to regions of high T2 signal on the FLAIR images. (F) There is a small focus of blooming within the left occipital lobe, in keeping with petechial haemorrhage. FLAIR, fluid-attenuated inversion recovery; PRES, posterior reversible encephalopathy syndrome.

    Brain imaging shows bilateral cortical–subcortical vasogenic oedema that falls into three anatomical patterns seen in about 70% of patients: a dominant parieto-occipital pattern (22%), a holohemispheric watershed pattern (23%); and superior frontal sulcus pattern (27%).4 32 A central-variant (brainstem) pattern has also been identified, affecting the brainstem, basal ganglia, posterior limb of the internal capsule, cerebellum and periventricular regions, but has no cortical or subcortical involvement.33 PRES may rarely even involve the spinal cord.34 Frontal and temporal lobe involvement can occur in up to 75% of cases,4 but in practice, these patterns are commonly mixed; PRES with such combined patterns is actually more common than PRES with isolated parieto-occipital involvement.4 35

    Intracranial haemorrhages are found on imaging in 10%–25% of cases, mostly intraparenchymal or subarachnoid.31 36 Up to 65% of patients who undergo follow-up MRI with susceptibility-weighted imaging have microhaemorrhages.31 PRES-associated intracranial haemorrhage probably results either from reperfusion injury in the setting of vasoconstriction or from pial vessel rupture secondary to severe hypertension.37

    PRES and RCVS are well known to overlap, and so cerebral CT or MR angiography is recommended to help to distinguish them.15 16 In RCVS, vessel wall MR angiography shows arterial wall thickening but without arterial wall enhancement, consistent with transient vasoconstriction.38 However, PRES occurs in 9%–38% of RCVS cases,15–18 and 87% of PRES cases have angiographic changes consistent with RCVS, such as diffuse vasoconstriction, focal vasculopathy or vessel pruning.39 Therefore, it is conceivable that PRES and RCVS are divergent phenotypical manifestations of a shared pathophysiology.20

    Laboratory investigations should be guided by the clinical context. It is reasonable to take an initial blood panel of full blood count, renal function, electrolytes, liver function tests, ammonia and a urine toxicology screen. Patients with significant unexplained hypertension need specific testing for secondary causes of hypertension, such as a phaeochromocytoma, Conn’s syndrome or Cushing’s syndrome. If clinically indicated, and given the association of PRES with autoimmune disease, it is worth considering a serum vasculitis screen including antinuclear antibody, antineutrophil cytoplasmic antibody, extractable nuclear antigens, rheumatoid factor, complement, lupus anticoagulant, anticardiolipin antibodies, paraproteins, ACE, HIV serology, erythrocyte sedimentation rate and C reactive protein. Cerebrospinal fluid (CSF) examination is usually not necessary in clear cases of PRES, unless atypical MRI patterns make it important to exclude an alternative diagnosis such as encephalitis or demyelination (figure 2). Obviously a lumbar puncture should not be performed if there are signs of raised intracranial pressure.

    Figure 2
    Figure 2

    Imaging findings in atypical PRES. MR scan of the brain of a 45-year-old woman with PRES and a history of multiple sclerosis who presented with reduced level of consciousness. (A,B) Axial FLAIR sequence shows extensive brainstem, cerebellar, bilateral thalami, corpus callosum and bilateral anterior temporal lobe hyperintense signal change. (C) Sagittal FLAIR sequence shows extensive brainstem and corpus callosum hyperintensities. (D) Coronal FLAIR showing confluent brainstem, bilateral temporal lobe and left parietal lobe hyperintensities. (E,F) Areas of diffusion-weighted imaging hyperintensity and hyperintense apparent diffusion coefficient signal involving the midbrain and bilateral cerebellar hemispheres consistent with vasogenic oedema, which corresponds to regions of high T2 signal. FLAIR, fluid-attenuated inversion recovery; PRES, posterior reversible encephalopathy syndrome.

    Do I need to request further imaging?

    Most cases of PRES do not need formal digital subtraction angiography, but it is worth considering this in patients with possible RCVS or those in whom CT or MR angiography shows a possible intracranial arteriopathy. Digital subtraction angiography may identify typical features of RCVS or of cerebral vasculitis, leading to specific therapies for each, including calcium channel blockers for RCVS or immune therapy for cerebral vasculitis.

    Differential diagnoses of PRES

    Table 1 shows the numerous conditions that clinicians need to consider in the differential diagnosis of PRES. While not all of these would be mistaken for classic PRES, they share clinical and/or radiological features with PRES that could lead to diagnostic confusion. The most important differentials include viral and autoimmune encephalitis, demyelinating disease, toxic leucoencephalopathies, malignancy such as gliomatosis cerebri, CNS vasculitis, central/extrapontine myelinolysis and acute stroke, especially due to cerebral venous thrombosis.32 A history of prior radiation exposure might suggest stroke-like migraine attacks after radiation therapy syndrome. Differentiating PRES from these other conditions requires a thorough review of risk factors, additional targeted testing and follow-up imaging.32

    View this table:
    Table 1

    Imaging and other key findings distinguishing between differential diagnoses

    Management

    How do I approach the acute management of PRES?

    The acute management of PRES is supportive and includes removing or reversing any suspected cause, for example, correcting hypertension (table 2). There have been no randomised trials on the various interventions used to treat PRES, and treatment guidelines are generally by consensus opinion. It is essential to recognise the condition promptly and to remove or reverse the precipitating factor, including chemotherapy or an immunosuppressive agent.1 Patients need to be hydrated and to have any electrolyte disturbances corrected. Patients in whom cerebral oedema is causing raised intracranial pressure may require neurosurgical measures (see What about cases of malignant PRES? section)

    View this table:
    Table 2

    Acute therapies for PRES

    Patients with acute hypertension should have their blood pressure gradually reduced by no more 20%–25% in the first few hours to avoid the risk of cerebral, coronary and renal ischaemia.40 Clinicians should aim for a mean arterial pressure of between 105 and 125 mm Hg, and continuous intravenous infusions are often required. First-line antihypertensive agents include nicardipine (5–15 mg/hour), labetalol (2–3 mg/min) and nimodipine; second-line agents include sodium nitroprusside, hydralazine and diazoxide.41 Nitroglycerine is not recommended in patients with PRES as it may aggravate cerebral oedema.42 Table 1 details the antihypertensive agents recommended for managing PRES.

    Once PRES is suspected, clinicians should consider transfer to an intensive care setting, as up to 70% of patients ultimately require intensive care for symptom management,43 and 35%–40% require mechanical ventilation for 3–7 days.1 44 Indications for transfer to an intensive care unit setting include encephalopathy, seizures, ventilatory depression and the need for invasive blood pressure support.43

    Management of PRES in specific situations

    It is crucial for neurological improvement that patients with intraparenchymal or subarachnoid haemorrhage should have their blood pressure gradually reduced.45 Those with marked renal failure need prompt dialysis. Pregnant women should be considered for early delivery46 and should best avoid ACE inhibitors.

    Patients who develop status epilepticus require emergency management with benzodiazepines and loading doses of sodium valproate, levetiracetam or phenytoin (table 2). However, there are no standard guidelines for managing PRES-associated seizures without status epilepticus, and here treatment with antiseizure medications is decided on an individual basis. Epilepsy develops in only 1.0%–3.9% of patients who suffer a PRES-associated seizure,13 47 and so most patients do not need long-term antiseizure medications; clinicians should consider weaning and stopping these medications once the acute phase of PRES has resolved.

    What about cases of malignant PRES?

    Malignant PRES is defined based on clinical criteria consisting of a Glasgow Coma Scale (GCS) score of <8 and clinical decline despite standard medical management for elevated intracranial pressure. In addition, there must be radiological evidence of oedema or intracerebral haemorrhage exerting mass effect, such as effacement of basal cisterns, transtentorial, tonsillar or uncal herniation.48 Malignant PRES requires aggressive supportive care that may include mechanical ventilation, transfusion of blood products for reversal of coagulopathy and corticosteroids for those with autoimmune disorders. Patients with PRES whose GCS score is ≤8 should have intracranial pressure monitoring whenever possible.48 Various interventions undertaken in patients with raised intracranial pressure include osmotherapy, CSF drainage by external ventricular drain, craniectomy and evacuation of haematomas; these are reported to reduce mortality rates in comparison to historic reports.49 50 Patients with acute obstructive hydrocephalus may require an external ventricular drain.51

    Long-term management of PRES

    Retrospective studies suggest that recurrent PRES occurs in 4% of patients,52 especially in those with persistent risk factors such as sickle cell crises, autoimmune conditions, hypertensive crises, renal failure and mitochondrial disorders.53

    Although PRES was initially described as benign, wholly reversible and with a good prognosis, its mortality is around 19%, and about 44% of patients are left with varying degrees of functional impairments.3 54 Follow-up imaging may find residual structural lesions in 40% of cases.5

    Factors associated with poor outcomes include severe encephalopathy, a hypertensive cause, hyperglycaemia, a neoplastic cause, a longer time to control the causative factor, the presence of multiple comorbidities, elevated C reactive protein, low CSF glucose and coagulopathy.3 54–56 Various imaging features associated with poor outcome include corpus callosum involvement, extensive cerebral oedema or worsening imaging severity, intracerebral haemorrhage, subarachnoid haemorrhage and restrictive diffusion on imaging.30 55 57 58

    It is difficult to identify a single predictor of outcome in PRES. Several studies have found a correlation between the degree of hypertension and both the clinical outcome and severity of oedema on imaging, but the presence or patterns of gadolinium-based contrast enhancement does not correlate with functional outcomes.30

    The occurrence of seizures during the acute phase is not associated with increased length of hospital stay, morbidity, mortality or nursing home placement on discharge.13 14 43 Despite a high frequency of seizures during the acute phase, patients have only a low risk of long-term unprovoked seizures, and epilepsy is rare.13

    What do I need to know about the pathophysiology of PRES?

    The mechanism underlying the development of PRES is poorly understood. PRES is a disorder of dysregulated perfusion of the brain, typically resulting in reversible vasogenic oedema. There are several theories regarding the cerebral vasculature dysregulation in PRES, but no single mechanism explains the development of PRES in all cases.59 Thus, multiple non-exclusive mechanisms probably contribute, including excessive hypertension, impaired cerebral autoregulation causing cerebral hyperperfusion and endothelial dysfunction.32 The posterior circulation may be more vulnerable to hyperperfusion as it has less sympathetic innervation to counter reflex parasympathetic vasodilation.60 Patients with PRES commonly have acute fluctuations in blood pressure, but it is not known whether these are the cause or a secondary effect of the syndrome.32 61 Endothelial dysfunction may result, either directly or as a downstream effect, from the secretion of cytokines, including tumour necrosis factor alpha, interleukin-1, interferon gamma and vascular endothelial growth factor.62

    Conclusion

    PRES is a neurological condition that commonly presents with visual disturbance, seizures and encephalopathy, and has a wide range of causes including hypertension, pre-eclampsia, renal failure and immunosuppression. Its diagnosis is facilitated by the characteristic MR brain scan changes of posterior subcortical oedema. Its treatment involves correcting identifiable triggers and supporting patients through the acute phase of the illness. The prognosis is generally favourable but more severely affected patients require intensive care support and may be left with residual neurological deficits. Key areas for further research are to gain a better understanding of its pathophysiology and to obtain better data regarding the need for long-term treatment of seizures with antiseizure medications.

    Key points

    • Posterior reversible encephalopathy syndrome (PRES) most commonly presents with visual disturbance, seizures and altered consciousness.

    • PRES can occur in the context of hypertension, renal failure, autoimmune disease and with the use of immunosuppressive therapy or chemotherapy.

    • MR scan of the brain confirms the oedema, which is often, but not exclusively, in posterior cortical and subcortical structures.

    • The treatment of PRES can be challenging but should be implemented early to minimise the potential for significant morbidity and mortality that can occur in more severe cases.

    Further reading

    • Bartynski WS, Boardman JF. Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol. (2007) 28:1320–7.

    • Singhal AB, Hajj-Ali RA, Topcuoglu MA, et al. Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol 2011;68:1005–12.

    • Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions.Lancet Neurol.(2015) 14:914–25.

    Ethics statements

    Patient consent for publication

    Ethics approval

    This study does not involve human participants.

    References

    1. ↵
      2. Lee VH ,
      3. Wijdicks EFM ,
      4. Manno EM , et al
      . Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol 2008;65:205–10.doi:10.1001/archneurol.2007.46 pmid:http://www.ncbi.nlm.nih.gov/pubmed/18268188
      OpenUrlCrossRefPubMedWeb of Science
    2. ↵
      2. Fugate JE ,
      3. Claassen DO ,
      4. Cloft HJ , et al
      . Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc 2010;85:427–32.doi:10.4065/mcp.2009.0590 pmid:http://www.ncbi.nlm.nih.gov/pubmed/20435835
      OpenUrlCrossRefPubMedWeb of Science
    3. ↵
      2. Legriel S ,
      3. Schraub O ,
      4. Azoulay E , et al
      . Determinants of recovery from severe posterior reversible encephalopathy syndrome. PLoS One 2012;7:e44534.doi:10.1371/journal.pone.0044534 pmid:http://www.ncbi.nlm.nih.gov/pubmed/23024751
      OpenUrlCrossRefPubMed
    4. ↵
      2. Bartynski WS ,
      3. Boardman JF
      . Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome. American Journal of Neuroradiology 2007;28:1320–7.doi:10.3174/ajnr.A0549
      OpenUrlCrossRefPubMedWeb of Science
    5. ↵
      2. Liman TG ,
      3. Bohner G ,
      4. Heuschmann PU , et al
      . The clinical and radiological spectrum of posterior reversible encephalopathy syndrome: the retrospective Berlin preS study. J Neurol 2012;259:155–64.doi:10.1007/s00415-011-6152-4 pmid:http://www.ncbi.nlm.nih.gov/pubmed/21717193
      OpenUrlCrossRefPubMed
    6. ↵
      2. Brewer J ,
      3. Owens MY ,
      4. Wallace K , et al
      . Posterior reversible encephalopathy syndrome in 46 of 47 patients with eclampsia. Am J Obstet Gynecol 2013;208:468.e1–468.e6.doi:10.1016/j.ajog.2013.02.015 pmid:http://www.ncbi.nlm.nih.gov/pubmed/23395926
      OpenUrlPubMed
    7. ↵
      2. Reece DE ,
      3. Frei-Lahr DA ,
      4. Shepherd JD , et al
      . Neurologic complications in allogeneic bone marrow transplant patients receiving cyclosporin. Bone Marrow Transplant 1991;8:393–401.pmid:http://www.ncbi.nlm.nih.gov/pubmed/1768975
      OpenUrlPubMedWeb of Science
    8. ↵
      2. Bartynski WS ,
      3. Zeigler ZR ,
      4. Shadduck RK , et al
      . Pretransplantation conditioning influence on the occurrence of cyclosporine or FK-506 neurotoxicity in allogeneic bone marrow transplantation. AJNR Am J Neuroradiol 2004;25:261–9.pmid:http://www.ncbi.nlm.nih.gov/pubmed/14970028
      OpenUrlPubMedWeb of Science
    9. ↵
      2. Staykov D ,
      3. Schwab S
      . Posterior reversible encephalopathy syndrome. J Intensive Care Med 2012;27:11–24.doi:10.1177/0885066610393634 pmid:http://www.ncbi.nlm.nih.gov/pubmed/21257628
      OpenUrlCrossRefPubMed
    10. ↵
      2. Bartynski WS ,
      3. Tan HP ,
      4. Boardman JF , et al
      . Posterior reversible encephalopathy syndrome after solid organ transplantation. AJNR Am J Neuroradiol 2008a;29:924–30.doi:10.3174/ajnr.A0960 pmid:http://www.ncbi.nlm.nih.gov/pubmed/18272559
      OpenUrlPubMed
    11. ↵
      2. Canney M ,
      3. Kelly D ,
      4. Clarkson M
      . Posterior reversible encephalopathy syndrome in end-stage kidney disease: not strictly posterior or reversible. Am J Nephrol 2015;41:177–82.doi:10.1159/000381316 pmid:http://www.ncbi.nlm.nih.gov/pubmed/25871433
      OpenUrlPubMed
    12. ↵
      2. Lai C-C ,
      3. Chen W-S ,
      4. Chang Y-S , et al
      . Clinical features and outcomes of posterior reversible encephalopathy syndrome in patients with systemic lupus erythematosus. Arthritis Care Res 2013;65:1766–74.doi:10.1002/acr.22047
      OpenUrlCrossRefPubMed
    13. ↵
      2. Datar S ,
      3. Singh T ,
      4. Rabinstein AA , et al
      . Long-term risk of seizures and epilepsy in patients with posterior reversible encephalopathy syndrome. Epilepsia 2015;56:564–8.doi:10.1111/epi.12933 pmid:http://www.ncbi.nlm.nih.gov/pubmed/25690439
      OpenUrlPubMed
    14. ↵
      2. Kastrup O ,
      3. Gerwig M ,
      4. Frings M , et al
      . Posterior reversible encephalopathy syndrome (PRES): electroencephalographic findings and seizure patterns. J Neurol 2012;259:1383–9.doi:10.1007/s00415-011-6362-9 pmid:http://www.ncbi.nlm.nih.gov/pubmed/22189837
      OpenUrlCrossRefPubMed
    15. ↵
      2. Ducros A ,
      3. Boukobza M ,
      4. Porcher R , et al
      . The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130:3091–101.doi:10.1093/brain/awm256 pmid:http://www.ncbi.nlm.nih.gov/pubmed/18025032
      OpenUrlCrossRefPubMedWeb of Science
    16. ↵
      2. Ducros A ,
      3. Fiedler U ,
      4. Porcher R , et al
      . Hemorrhagic manifestations of reversible cerebral vasoconstriction syndrome: frequency, features, and risk factors. Stroke 2010;41:2505–11.doi:10.1161/STROKEAHA.109.572313 pmid:http://www.ncbi.nlm.nih.gov/pubmed/20884871
      OpenUrlAbstract/FREE Full Text
    17. ↵
      2. Singhal AB ,
      3. Hajj-Ali RA ,
      4. Topcuoglu MA , et al
      . Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol 2011;68:1005–12.doi:10.1001/archneurol.2011.68 pmid:http://www.ncbi.nlm.nih.gov/pubmed/21482916
      OpenUrlCrossRefPubMedWeb of Science
    18. ↵
      2. Chen S-P ,
      3. Fuh J-L ,
      4. Wang S-J , et al
      . Magnetic resonance angiography in reversible cerebral vasoconstriction syndromes. Ann Neurol 2010;67:648–56.doi:10.1002/ana.21951 pmid:http://www.ncbi.nlm.nih.gov/pubmed/20437562
      OpenUrlCrossRefPubMedWeb of Science
    19. ↵
      2. Bartynski WS ,
      3. Boardman JF
      . Catheter angiography, Mr angiography, and MR perfusion in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol 2008b;29:447–55.doi:10.3174/ajnr.A0839 pmid:http://www.ncbi.nlm.nih.gov/pubmed/18079186
      OpenUrlPubMed
    20. ↵
      2. Cappelen-Smith C ,
      3. Calic Z ,
      4. Cordato D
      . Reversible cerebral vasoconstriction syndrome: recognition and treatment. Curr Treat Options Neurol 2017;19:21.doi:10.1007/s11940-017-0460-7 pmid:http://www.ncbi.nlm.nih.gov/pubmed/28456915
      OpenUrlCrossRefPubMed
    21. ↵
      2. Rabinstein AA ,
      3. Mandrekar J ,
      4. Merrell R , et al
      . Blood pressure fluctuations in posterior reversible encephalopathy syndrome. J Stroke Cerebrovasc Dis 2012;21:254–8.doi:10.1016/j.jstrokecerebrovasdis.2011.03.011 pmid:http://www.ncbi.nlm.nih.gov/pubmed/21536456
      OpenUrlCrossRefPubMed
    22. ↵
      2. Casey SO ,
      3. Sampaio RC ,
      4. Michel E , et al
      . Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 2000;21:1199–206.pmid:http://www.ncbi.nlm.nih.gov/pubmed/10954269
      OpenUrlAbstract/FREE Full Text
    23. ↵
      2. Bartynski WS ,
      3. Boardman JF
      . Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol 2007;28:1320–7.doi:10.3174/ajnr.A0549 pmid:http://www.ncbi.nlm.nih.gov/pubmed/17698535
      OpenUrlCrossRefPubMedWeb of Science
    24. ↵
      2. Femia G ,
      3. Hardy TA ,
      4. Spies JM , et al
      . Posterior reversible encephalopathy syndrome following chemotherapy with oxaliplatin and a fluoropyrimidine: a case report and literature review. Asia Pac J Clin Oncol 2012;8:115–22.doi:10.1111/j.1743-7563.2012.01544.x pmid:http://www.ncbi.nlm.nih.gov/pubmed/22524570
      OpenUrlPubMed
    25. ↵
      2. Nagel S ,
      3. Köhrmann M ,
      4. Huttner HB , et al
      . Linezolid-induced posterior reversible leukoencephalopathy syndrome. Arch Neurol 2007;64:746–8.doi:10.1001/archneur.64.5.746 pmid:http://www.ncbi.nlm.nih.gov/pubmed/17502475
      OpenUrlCrossRefPubMed
    26. ↵
      2. Belmouaz S ,
      3. Desport E ,
      4. Leroy F , et al
      . Posterior reversible encephalopathy induced by intravenous immunoglobulin. Nephrol Dial Transplant 2008;23:417–9.doi:10.1093/ndt/gfm594 pmid:http://www.ncbi.nlm.nih.gov/pubmed/17971381
      OpenUrlCrossRefPubMedWeb of Science
    27. ↵
      2. Legriel S ,
      3. Bruneel F ,
      4. Spreux-Varoquaux O , et al
      . Lysergic acid amide-induced posterior reversible encephalopathy syndrome with status epilepticus. Neurocrit Care 2008;9:247–52.doi:10.1007/s12028-008-9096-5 pmid:http://www.ncbi.nlm.nih.gov/pubmed/18446448
      OpenUrlCrossRefPubMed
    28. ↵
      2. Dasari V ,
      3. Donohoe CD
      . Cocaine-Induced posterior reversible encephalopathy syndrome (PRES): a case report and review of the literature. J Neuroimaging Psychiatry Neurol 2018;03.doi:10.17756/jnpn.2018-025
    29. ↵
      2. Porcello Marrone LC ,
      3. Marrone BF ,
      4. Neto FK , et al
      . Posterior reversible encephalopathy syndrome following a scorpion sting. J Neuroimaging 2013;23:535–6.doi:10.1111/jon.12017 pmid:http://www.ncbi.nlm.nih.gov/pubmed/23551823
      OpenUrlPubMed
    30. ↵
      2. Karia SJ ,
      3. Rykken JB ,
      4. McKinney ZJ , et al
      . Utility and significance of gadolinium-based contrast enhancement in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol 2016;37:415–22.doi:10.3174/ajnr.A4563 pmid:http://www.ncbi.nlm.nih.gov/pubmed/26564441
      OpenUrlAbstract/FREE Full Text
    31. ↵
      2. McKinney AM ,
      3. Sarikaya B ,
      4. Gustafson C , et al
      . Detection of microhemorrhage in posterior reversible encephalopathy syndrome using susceptibility-weighted imaging. AJNR Am J Neuroradiol 2012;33:896–903.doi:10.3174/ajnr.A2886 pmid:http://www.ncbi.nlm.nih.gov/pubmed/22241378
      OpenUrlAbstract/FREE Full Text
    32. ↵
      2. Fugate JE ,
      3. Rabinstein AA
      . Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol 2015;14:914–25.doi:10.1016/S1474-4422(15)00111-8 pmid:http://www.ncbi.nlm.nih.gov/pubmed/26184985
      OpenUrlCrossRefPubMed
    33. ↵
      2. Ollivier M ,
      3. Bertrand A ,
      4. Clarençon F , et al
      . Neuroimaging features in posterior reversible encephalopathy syndrome: a pictorial review. J Neurol Sci 2017;373:188–200.doi:10.1016/j.jns.2016.12.007 pmid:http://www.ncbi.nlm.nih.gov/pubmed/28131186
      OpenUrlPubMed
    34. ↵
      2. de Havenon A ,
      3. Joos Z ,
      4. Longenecker L , et al
      . Posterior reversible encephalopathy syndrome with spinal cord involvement. Neurology 2014;83:2002–6.doi:10.1212/WNL.0000000000001026 pmid:http://www.ncbi.nlm.nih.gov/pubmed/25355822
      OpenUrlCrossRefPubMed
    35. ↵
      2. Tetsuka S ,
      3. Ogawa T
      . Posterior reversible encephalopathy syndrome: a review with emphasis on neuroimaging characteristics. J Neurol Sci 2019;404:72–9.doi:10.1016/j.jns.2019.07.018 pmid:http://www.ncbi.nlm.nih.gov/pubmed/31349066
      OpenUrlPubMed
    36. ↵
      2. Sharma A ,
      3. Whitesell RT ,
      4. Moran KJ
      . Imaging pattern of intracranial hemorrhage in the setting of posterior reversible encephalopathy syndrome. Neuroradiology 2010;52:855–63.doi:10.1007/s00234-009-0632-6 pmid:http://www.ncbi.nlm.nih.gov/pubmed/19956935
      OpenUrlCrossRefPubMedWeb of Science
    37. ↵
      2. Doss-Esper CE ,
      3. Singhal AB ,
      4. Smith MSA , et al
      . Reversible posterior leukoencephalopathy, cerebral vasoconstriction, and strokes after intravenous immune globulin therapy in Guillain-Barre syndrome. J Neuroimaging 2005;15:188–92.doi:10.1177/1051228404273820 pmid:http://www.ncbi.nlm.nih.gov/pubmed/15746232
      OpenUrlCrossRefPubMedWeb of Science
    38. ↵
      2. Mandell DM ,
      3. Matouk CC ,
      4. Farb RI , et al
      . Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: preliminary results. Stroke 2012;43:860–2.doi:10.1161/STROKEAHA.111.626184 pmid:http://www.ncbi.nlm.nih.gov/pubmed/22156692
      OpenUrlAbstract/FREE Full Text
    39. ↵
      2. Bartynski WS
      . Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol 2008b;29:1043–9.doi:10.3174/ajnr.A0929 pmid:http://www.ncbi.nlm.nih.gov/pubmed/18403560
      OpenUrlPubMed
    40. ↵
      2. Williams B ,
      3. Mancia G ,
      4. Spiering W , et al
      . 2018 ESC/ESH guidelines for the management of arterial hypertension: the task force for the management of arterial hypertension of the European Society of cardiology and the European Society of hypertension: the task force for the management of arterial hypertension of the European Society of cardiology and the European Society of hypertension. J Hypertens 2018;36:1953–2041.doi:10.1097/HJH.0000000000001940 pmid:http://www.ncbi.nlm.nih.gov/pubmed/30234752
      OpenUrlCrossRefPubMed
    41. ↵
      2. Servillo G ,
      3. Bifulco F ,
      4. De Robertis E , et al
      . Posterior reversible encephalopathy syndrome in intensive care medicine. Intensive Care Med 2007;33:230–6.doi:10.1007/s00134-006-0459-0 pmid:http://www.ncbi.nlm.nih.gov/pubmed/17119920
      OpenUrlCrossRefPubMedWeb of Science
    42. ↵
      2. Finsterer J ,
      3. Schlager T ,
      4. Kopsa W , et al
      . Nitroglycerin-aggravated pre-eclamptic posterior reversible encephalopathy syndrome (PRES). Neurology 2003;61:715–6.doi:10.1212/01.WNL.0000080369.87484.06 pmid:http://www.ncbi.nlm.nih.gov/pubmed/12963776
      OpenUrlPubMed
    43. ↵
      2. Hinduja A ,
      3. Habetz K ,
      4. Raina SK , et al
      . Predictors of intensive care unit utilization in patients with posterior reversible encephalopathy syndrome. Acta Neurol Belg 2017;117:201–6.doi:10.1007/s13760-016-0703-5 pmid:http://www.ncbi.nlm.nih.gov/pubmed/27680733
      OpenUrlPubMed
    44. ↵
      2. Li R ,
      3. Mitchell P ,
      4. Dowling R , et al
      . Is hypertension predictive of clinical recurrence in posterior reversible encephalopathy syndrome? J Clin Neurosci 2013;20:248–52.doi:10.1016/j.jocn.2012.02.023 pmid:http://www.ncbi.nlm.nih.gov/pubmed/23219827
      OpenUrlCrossRefPubMed
    45. ↵
      2. Dhar R ,
      3. Dacey R ,
      4. Human T , et al
      . Unilateral posterior reversible encephalopathy syndrome with hypertensive therapy of contralateral vasospasm: case report. Neurosurgery 2011;69:1.doi:10.1227/NEU.0b013e318223b995 pmid:http://www.ncbi.nlm.nih.gov/pubmed/21971491
      OpenUrlCrossRefPubMed
    46. ↵
      2. Ekawa Y ,
      3. Shiota M ,
      4. Tobiume T , et al
      . Reversible posterior leukoencephalopathy syndrome accompanying eclampsia: correct diagnosis using preoperative MRI. Tohoku J Exp Med 2012;226:55–8.doi:10.1620/tjem.226.55 pmid:http://www.ncbi.nlm.nih.gov/pubmed/22186037
      OpenUrlPubMed
    47. ↵
      2. Heo K ,
      3. Cho KH ,
      4. Lee MK , et al
      . Development of epilepsy after posterior reversible encephalopathy syndrome. Seizure 2016;34:90–4.doi:10.1016/j.seizure.2015.12.005 pmid:http://www.ncbi.nlm.nih.gov/pubmed/26760446
      OpenUrlPubMed
    48. ↵
      2. Akins PT ,
      3. Axelrod Y ,
      4. Silverthorn JW , et al
      . Management and outcomes of malignant posterior reversible encephalopathy syndrome. Clin Neurol Neurosurg 2014;125:52–7.doi:10.1016/j.clineuro.2014.06.034 pmid:http://www.ncbi.nlm.nih.gov/pubmed/25086431
      OpenUrlPubMed
    49. ↵
      2. Hefzy HM ,
      3. Bartynski WS ,
      4. Boardman JF , et al
      . Hemorrhage in posterior reversible encephalopathy syndrome: imaging and clinical features. AJNR Am J Neuroradiol 2009;30:1371–9.doi:10.3174/ajnr.A1588 pmid:http://www.ncbi.nlm.nih.gov/pubmed/19386731
      OpenUrlAbstract/FREE Full Text
    50. ↵
      2. Aranas RM ,
      3. Prabhakaran S ,
      4. Lee VH
      . Posterior reversible encephalopathy syndrome associated with hemorrhage. Neurocrit Care 2009;10:306–12.doi:10.1007/s12028-009-9200-5 pmid:http://www.ncbi.nlm.nih.gov/pubmed/19225908
      OpenUrlCrossRefPubMedWeb of Science
    51. ↵
      2. Catherine C ,
      3. Yanta C ,
      4. Saand AR , et al
      . Pearls & Oy-sters: The dangers of PRES: An atypical case with life-threatening presentation. Neurology 2019;92:e282–5.doi:10.1212/WNL.0000000000006775 pmid:http://www.ncbi.nlm.nih.gov/pubmed/30643036
      OpenUrlPubMed
    52. ↵
      2. Sweany JM ,
      3. Bartynski WS ,
      4. Boardman JF
      . "Recurrent" posterior reversible encephalopathy syndrome: report of 3 cases--PRES can strike twice! J Comput Assist Tomogr 2007;31:148–56.doi:10.1097/01.rct.0000233127.21303.b9 pmid:http://www.ncbi.nlm.nih.gov/pubmed/17259848
      OpenUrlCrossRefPubMedWeb of Science
    53. ↵
      2. Finsterer J ,
      3. Stöllberger C ,
      4. Ostermann E , et al
      . Recurrent posterior reversible encephalopathy syndrome in mitochondrial disorder. Blood Press 2009;18:126–9.doi:10.1080/08037050902975106 pmid:http://www.ncbi.nlm.nih.gov/pubmed/19437173
      OpenUrlPubMed
    54. ↵
      2. Alhilali LM ,
      3. Reynolds AR ,
      4. Fakhran S
      . A multi-disciplinary model of risk factors for fatal outcome in posterior reversible encephalopathy syndrome. J Neurol Sci 2014;347:59–65.doi:10.1016/j.jns.2014.09.019 pmid:http://www.ncbi.nlm.nih.gov/pubmed/25271189
      OpenUrlCrossRefPubMed
    55. ↵
      2. Siebert E ,
      3. Bohner G ,
      4. Liebig T , et al
      . Factors associated with fatal outcome in posterior reversible encephalopathy syndrome: a retrospective analysis of the Berlin preS study. J Neurol 2017;264:237–42.doi:10.1007/s00415-016-8328-4 pmid:http://www.ncbi.nlm.nih.gov/pubmed/27815684
      OpenUrlPubMed
    56. ↵
      2. Liman TG ,
      3. Bohner G ,
      4. Endres M , et al
      . Discharge status and in-hospital mortality in posterior reversible encephalopathy syndrome. Acta Neurol Scand 2014;130:34–9.doi:10.1111/ane.12213 pmid:http://www.ncbi.nlm.nih.gov/pubmed/24329761
      OpenUrlPubMed
    57. ↵
      2. Chen Z ,
      3. Zhang G ,
      4. Lerner A , et al
      . Risk factors for poor outcome in posterior reversible encephalopathy syndrome: systematic review and meta-analysis. Quant Imaging Med Surg 2018;8:421–32.doi:10.21037/qims.2018.05.07 pmid:http://www.ncbi.nlm.nih.gov/pubmed/29928607
      OpenUrlPubMed
    58. ↵
      2. Schweitzer AD ,
      3. Parikh NS ,
      4. Askin G , et al
      . Imaging characteristics associated with clinical outcomes in posterior reversible encephalopathy syndrome. Neuroradiology 2017;59:379–86.doi:10.1007/s00234-017-1815-1 pmid:http://www.ncbi.nlm.nih.gov/pubmed/28289809
      OpenUrlCrossRefPubMed
    59. ↵
      2. Feske SK
      . Posterior reversible encephalopathy syndrome: a review. Semin Neurol 2011;31:202–15.doi:10.1055/s-0031-1277990 pmid:http://www.ncbi.nlm.nih.gov/pubmed/21590625
      OpenUrlCrossRefPubMed
    60. ↵
      2. Roloff EVL ,
      3. Tomiak-Baquero AM ,
      4. Kasparov S , et al
      . Parasympathetic innervation of vertebrobasilar arteries: is this a potential clinical target? J Physiol 2016;594:6463–85.doi:10.1113/JP272450 pmid:http://www.ncbi.nlm.nih.gov/pubmed/27357059
      OpenUrlPubMed
    61. ↵
      2. Bartynski WS
      . Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features. AJNR Am J Neuroradiol 2008c;29:1036–42.doi:10.3174/ajnr.A0928 pmid:http://www.ncbi.nlm.nih.gov/pubmed/18356474
      OpenUrlPubMed
    62. ↵
      2. Marra A ,
      3. Vargas M ,
      4. Striano P , et al
      . Posterior reversible encephalopathy syndrome: the endothelial hypotheses. Med Hypotheses 2014;82:619–22.doi:10.1016/j.mehy.2014.02.022 pmid:http://www.ncbi.nlm.nih.gov/pubmed/24613735
      OpenUrlCrossRefPubMed

    Footnotes

    • Contributors JDT, AGK and TH undertook design of the article, drafting of the article and critical review of the article. MAK undertook drafting of the article and critical review of the article.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Commissioned. Externally peer reviewed by Aaron Berkowitz, Pasadena, California, USA.

    Linked Articles

    • Editors’ commentary
      Highlights from this issue
      Phil E M Smith Geraint N Fuller
      Practical Neurology 2022; 22 175-175 Published Online First: 17 May 2022. doi: 10.1136/practneurol-2022-003476

    Other content recommended for you