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Review Article| Volume 404, P72-79, September 15, 2019

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Posterior reversible encephalopathy syndrome: A review with emphasis on neuroimaging characteristics

      Highlights

      • Typical MRI findings of PRES in parieto-occipital lobes caused by vasogenic edema.
      • Atypical PRES cases show brainstem, basal ganglia, and cerebellum.
      • Endothelial dysfunction may be the predominant pathophysiological mechanism of PRES.
      • MRI findings have been useful for the prompt diagnosis of PRES.
      • MRI findings play a central role in constructing the algorithm.

      Abstract

      Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological condition that involves the acute onset of headache, confusion, optical impairments, and seizures with accompanying vasogenic edema on brain imaging. PRES is a complex disorder with many causative factors, including underlying conditions such as hypertensive encephalopathy, eclampsia, collagen disease, and severe infection. Although the exact pathophysiological mechanism is not completely understood and remains controversial, the predominant proposed mechanism is endothelial dysfunction, which is preceded by hypertension, immunosuppressive agents, or cytotoxic medication. Magnetic resonance imaging (MRI) facilitates prompt diagnosis and treatment and leads to good outcomes. Findings are characterized by the following: hyperintensity on fluid-attenuated inversion recovery images and apparent diffusion coefficient mapping, and isointensity on diffusion weighted images involving the parieto-occipital or posterior frontal cortical–subcortical regions that are recognized in >90% of patients, and reversibility of neuroimaging abnormalities, with the latter being the most important. As an algorithm to standardize the diagnosis of PRES has not yet been developed, this review presents a diagnostic algorithm based on the types of MRI findings. This algorithm may provide a better understanding of the characteristics that compose PRES and bring us one step closer to the standardization of PRES diagnosis, helping clinicians evaluate individual features while also considering competing differential diagnoses.

      Keywords

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      References

        • Hinchey J.
        • Chaves C.
        • Appignani B.
        • et al.
        A reversible posterior leukoencephalopathy syndrome.
        N. Engl. J. Med. 1996; 334: 494-500
        • Fugate J.E.
        • Rabinstein A.A.
        Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions.
        Lancet Neurol. 2015; 14: 914-925
        • Liman T.G.
        • Bohner G.
        • Heuschmann P.U.
        • et al.
        The clinical and radiological spectrum of posterior reversible encephalopathy syndrome: the retrospective Berlin PRES study.
        J. Neurol. 2012; 259: 155-164
        • Lee V.H.
        • Wijdicks E.F.
        • Manno E.M.
        • et al.
        Clinical spectrum of reversible posterior leukoencephalopathy syndrome.
        Arch. Neurol. 2008; 65: 205-210
        • Fischer M.
        • Schmutzhard E.
        Posterior reversible encephalopathy syndrome.
        J. Neurol. 2017; 264: 1608-1616
        • Bartynski W.S.
        Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema.
        AJNR Am. J. Neuroradiol. 2008; 29: 1043-1049
        • Benigni A.
        • Morigi M.
        • Perico N.
        • et al.
        The acute effect of fk506 and cyclosporine on endothelial cell function and renal vascular resistance.
        Transplantation. 1992; 54: 775-780
        • Bartynski W.S.
        • Boardman J.F.
        • Zeigler Z.R.
        • et al.
        Posterior reversible encephalopathy syndrome in infection, sepsis, and shock.
        AJNR Am. J. Neuroradiol. 2006; 27: 2179-2190
        • Lamy C.
        • Oppenheim C.
        • Mas J.L.
        Posterior reversible encephalopathy syndrome.
        Handb. Clin. Neurol. 2014; 121: 1687-1701
        • Mayama M.
        • Uno K.
        • Tano S.
        • et al.
        Incidence of posterior reversible encephalopathy syndrome in eclamptic and patients with preeclampsia with neurologic symptoms.
        Am. J. Obstet. Gynecol. 2016; 215: e231-e239
        • Marra A.
        • Vargas M.
        • Striano P.
        • et al.
        Posterior reversible encephalopathy syndrome: the endothelial hypotheses.
        Med. Hypotheses. 2014; 82: 619-622
        • Feske S.K.
        Posterior reversible encephalopathy syndrome: a review.
        Semin. Neurol. 2011; 31: 202-215
        • Allen M.L.
        • Kulik T.
        • Keyrouz S.G.
        • Dhar R.
        Posterior reversible encephalopathy syndrome as a complication of induced hypertension in subarachnoid hemorrhage: a case-control study.
        Neurosurgery. 2019; 85: 223-230
        • Bartynski W.S.
        Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features.
        AJNR Am. J. Neuroradiol. 2008; 29: 1036-1042
        • Kastrup O.
        • Gerwig M.
        • Frings M.
        • et al.
        Posterior reversible encephalopathy syndrome (PRES): electroencephalographic findings and seizure patterns.
        J. Neurol. 2012; 259: 1383-1389
        • Ay H.
        • Buonanno F.S.
        • Schaefer P.W.
        • et al.
        Posterior leukoencephalopathy without severe hypertension: utility of diffusion-weighted MRI.
        Neurology. 1998; 51: 1369-1376
        • Fugate J.E.
        • Claassen D.O.
        • Cloft H.J.
        • et al.
        Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings.
        Mayo Clin. Proc. 2010; 85: 427-432
        • Tajima Y.
        • Isonishi K.
        • Kashiwaba T.
        • et al.
        Two similar cases of encephalopathy, possibly a reversible posterior leukoencephalopathy syndrome: serial findings of magnetic resonance imaging, SPECT and angiography.
        Intern. Med. 1999; 38: 54-58
        • Schweitzer A.D.
        • Parikh N.S.
        • Askin G.
        • et al.
        Imaging characteristics associated with clinical outcomes in posterior reversible encephalopathy syndrome.
        Neuroradiology. 2017; 59: 379-386
        • Rykken J.B.
        • McKinney A.M.
        Posterior reversible encephalopathy syndrome.
        Semin. Ultrasound CT MR. 2014; 35: 118-135
        • Bartynski W.S.
        • Boardman J.F.
        Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome.
        AJNR Am. J. Neuroradiol. 2007; 28: 1320-1327
        • McKinney A.M.
        • Jagadeesan B.D.
        • Truwit C.L.
        Central-variant posterior reversible encephalopathy syndrome: brainstem or basal ganglia involvement lacking cortical or subcortical cerebral edema.
        AJR Am. J. Roentgenol. 2013; 201: 631-638
        • Datar S.
        • Singh T.D.
        • Fugate J.E.
        • Mandrekar J.
        • Rabinstein A.A.
        • Hocker S.
        Albuminocytologic dissociation in posterior reversible encephalopathy syndrome.
        Mayo Clin. Proc. 2015; 90: 1366-1371
        • Neeb L.
        • Hoekstra J.
        • Endres M.
        • Siegerink B.
        • Siebert E.
        • Liman T.G.
        Spectrum of cerebral spinal fluid findings in patients with posterior reversible encephalopathy syndrome.
        J. Neurol. 2016; 263: 30-34
        • Ellis C.A.
        • McClelland A.C.
        • Mohan S.
        • et al.
        Cerebrospinal fluid in posterior reversible encephalopathy syndrome: implications of elevated protein and pleocytosis.
        Neurohospitalist. 2019; 9: 58-64
        • Roth C.
        • Ferbert A.
        Posterior reversible encephalopathy syndrome: long-term follow-up.
        J. Neurol. Neurosurg. Psychiatry. 2010; 81: 773-777
        • Sha Z.
        • Moran B.P.
        • McKinney 4th, A.M.
        • Henry T.R.
        Seizure outcomes of posterior reversible encephalopathy syndrome and correlations with electroencephalographic changes.
        Epilepsy Behav. 2015; 48: 70-74
        • Bastide L.
        • Legros B.
        • Rampal N.
        • Gilmore E.J.
        • Hirsch L.J.
        • Gaspard N.
        Clinical correlates of periodic discharges and nonconvulsive seizures in posterior reversible encephalopathy syndrome (PRES).
        Neurocrit. Care. 2018; 29: 481-490
        • Provenzale J.M.
        • Petrella J.R.
        • Cruz L.C.
        • et al.
        Quantitative assessment of diffusion abnormalities in posterior reversible encephalopathy syndrome.
        AJNR Am. J. Neuroradiol. 2001; 22: 1455-1461
        • Covarrubias D.J.
        • Luetmer P.H.
        • Campeau N.G.
        • et al.
        Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images.
        AJNR Am. J. Neuroradiol. 2002; 23: 1038-1048
        • Bartynski W.S.
        • Boardman J.F.
        Catheter angiography, MR angiography, and MR perfusion in posterior reversible encephalopathy syndrome.
        AJNR Am. J. Neuroradiol. 2008; 29: 447-455
        • Gao B.
        • Liu F.L.
        • Zhao B.
        • et al.
        Association of degree and type of edema in posterior reversible encephalopathy syndrome with serum lactate dehydrogenase level: initial experience.
        Eur. J. Radiol. 2012; 81: 2844-2847
        • Legriel S.
        • Schraub O.
        • Azoulay E.
        • et al.
        Determinants of recovery from severe posterior reversible encephalopathy syndrome.
        PLoS ONE. 2012; 7e44534
        • Sha Z.
        • Moran B.P.
        • McKinney A.M.
        • et al.
        Seizure outcomes of posterior reversible encephalopathy syndrome and correlations with electroencephalographic changes.
        Epilepsy Behav. 2015; 48: 70-74
        • Heo K.
        • Cho K.H.
        • Lee M.K.
        • et al.
        Development of epilepsy after posterior reversible encephalopathy syndrome.
        Seizure. 2016; 34: 90-94
        • McKinney A.M.
        • Short J.
        • Truwit C.L.
        • et al.
        Posterior reversible encephalopathy syndrome: incidence of atypical regions of involvement and imaging findings.
        AJR Am. J. Roentgenol. 2007; 189: 904-912
        • Gao B.
        • Lyu C.
        • Lerner A.
        • McKinney A.M.
        Controversy of posterior reversible encephalopathy syndrome: what have we learnt in the last 20 years?.
        J. Neurol. Neurosurg. Psychiatry. 2018; 89: 14-20
        • Tetsuka S.
        • Nonaka H.
        Importance of correctly interpreting magnetic resonance imaging to diagnose posterior reversible encephalopathy syndrome associated with HELLP syndrome: a case report.
        BMC Med. Imaging. 2017; 17: 35
        • Casey S.O.
        • Sampaio R.C.
        • Michel E.
        • et al.
        Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions.
        Am. J. Neuroradiol. 2000; 21: 1199-1206
        • Jarosz J.M.
        • Howlett D.C.
        • Cox T.C.
        • et al.
        Cyclosporine-related reversible posterior leukoencephalopathy: MRI.
        Neuroradiology. 1997; 39: 711-715
        • Casey S.O.
        • Truwit C.L.
        Pontine reversible edema: a newly recognized imaging variant of hypertensive encephalopathy?.
        Am. J. Neuroradiol. 2000; 21: 243-245
        • de Seze J.
        • Mastain B.
        • Stojkovic T.
        • et al.
        Unusual MR findings of the brain stem in arterial hypertension.
        Am. J. Neuroradiol. 2000; 21: 391-394
        • Chang G.Y.
        • Keane J.R.
        Hypertensive brainstem encephalopathy: three cases presenting with severe brainstem edema.
        Neurology. 1999; 53: 652-654
        • Burnett M.M.
        • Hess C.P.
        • Roberts J.P.
        • et al.
        Josephson SA. Presentation of reversible posterior leukoencephalopathy syndrome in patients on calcineurin inhibitors.
        Clin. Neurol. Neurosurg. 2010; 112: 886-891
        • Moon S.N.
        • Jeon S.J.
        • Choi S.S.
        • et al.
        Can clinical and MRI findings predict the prognosis of variant and classical type of posterior reversible encephalopathy syndrome (PRES)?.
        Acta Radiol. 2013; 54: 1182-1190
        • Liman T.G.
        • Bohner G.
        • Endres M.
        • et al.
        Discharge status and in-hospital mortality in posterior reversible encephalopathy syndrome.
        Acta Neurol. Scand. 2014; 130: 34-39
        • Shaharir S.S.
        • Remli R.
        • Marwan A.A.
        • et al.
        Posterior reversible encephalopathy syndrome in systemic lupus erythematosus: pooled analysis of the literature reviews and report of six new cases.
        Lupus. 2013; 22: 492-496
        • Schweitzer A.D.
        • Parikh N.S.
        • Askin G.
        • et al.
        Imaging characteristics associated with clinical outcomes in posterior reversible encephalopathy syndrome.
        Neuroradiology. 2017; 59: 379-386
        • Liman T.G.
        • Siebert E.
        • Endres M.
        Posterior reversible encephalopathy syndrome.
        Curr. Opin. Neurol. 2019; 32: 25-35
        • Vincent A.
        Developments in autoimmune channelopathies.
        Autoimmun. Rev. 2013; 12: 678-681
        • Ban S.P.
        • Hwang G.
        • Kim C.H.
        • Kwon O.K.
        Reversible cerebral vasoconstriction syndrome combined with posterior reversible encephalopathy syndrome after heart transplantation.
        J. Clin. Neurosci. 2017; 42: 118-121
        • Schaefer P.W.
        • Buonanno F.S.
        • Gonzalez R.G.
        • et al.
        Diffusion-weighted imaging discriminates between cytotoxic and vasogenic edema in a patient with eclampsia.
        Stroke. 1997; 28: 1082-1085
        • Shimono T.
        • Miki Y.
        • Toyoda H.
        • et al.
        MR imaging with quantitative diffusion mapping of tacrolimus-induced neurotoxicity in organ transplant patients.
        Eur. Radiol. 2003; 13: 986-993
        • 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: 1068-1070