Download PDF
Semin Neurol 2011; 31(2): 202-215
DOI: 10.1055/s-0031-1277990
© Thieme Medical Publishers

Posterior Reversible Encephalopathy Syndrome: A Review

Steven K. Feske1 , 2
  • 1Harvard Medical School, Brigham and Women's Hospital, Boston, Massachusetts
  • 2Division of Stroke, Department of Neurology, Brigham and Women's Hospital, Boston, Massachusetts
Further Information

Publication History

Publication Date:
17 May 2011 (online)

    Permissions and Reprints

ABSTRACT

Encephalopathy due to reversible cerebral edema is an important cause of neurologic morbidity accompanying many disorders. Although controversy remains concerning the pathophysiologic trigger, the mechanism of this disorder ultimately depends on failure of the blood–brain barrier to maintain the compartmentalization of intravascular fluid. This failure of the blood–brain barrier depends primarily on the capillary hydrostatic pressure, under the influence of the systemic blood pressure, and on the integrity of the structures that make up the blood–brain barrier, most importantly the vascular endothelium, under the influence of various diseases and toxic medications. Although typical clinical contexts and presentations have been well defined, many patients have atypical features that pose a diagnostic challenge. Therefore, awareness of this clinical variability is important for prompt diagnosis. This review discusses the history and pathophysiology of posterior reversible encephalopathy syndrome and then addresses its clinical diagnosis and management.

KEYWORDS

Hypertensive encephalopathy - posterior reversible encephalopathy syndrome - blood–brain barrier - cerebral autoregulation - endothelial dysfunction

REFERENCES

  • 1 Casey S O, Sampaio R C, Michel E, Truwit C L. 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 (7) 1199-1206
    PubMedGoogle Scholar
  • 2 Oppenheimer B S, Fishberg A M. Hypertensive encephalopathy.  Arch Intern Med. 1928;  41 264-278
    CrossrefPubMedGoogle Scholar
  • 3 Hinchey J, Chaves C, Appignani B et al.. A reversible posterior leukoencephalopathy syndrome.  N Engl J Med. 1996;  334 (8) 494-500
    CrossrefPubMedGoogle Scholar
  • 4 Dillon W P, Rowley H. The reversible posterior cerebral edema syndrome.  AJNR Am J Neuroradiol. 1998;  19 (3) 591
    PubMedGoogle Scholar
  • 5 Chesley L C. Hypertensive disorders in pregnancy. New York: Appleton-Centery-Crofts; 1978
    Google Scholar
  • 6 Luft F C, Dietz R. Franz Volhard in historical perspective.  Hypertension. 1993;  22 (2) 253-256
    PubMedGoogle Scholar
  • 7 Byrom F B. The pathogenesis of hypertensive encephalopathy and its relation to the malignant phase of hypertension; experimental evidence from the hypertensive rat.  Lancet. 1954;  267 (6831) 201-211
    PubMedGoogle Scholar
  • 8 Tamaki K, Sadoshima S, Baumbach G L, Iadecola C, Reis D J, Heistad D D. Evidence that disruption of the blood-brain barrier precedes reduction in cerebral blood flow in hypertensive encephalopathy.  Hypertension. 1984;  6 (2 Pt 2) I75-I81
    PubMedGoogle Scholar
  • 9 Adams R D, Vander Eecken H M. Vascular diseases of the brain.  Annu Rev Med. 1953;  4 213-252
    CrossrefPubMedGoogle Scholar
  • 10 McDowell F H. Cerebral ischemia and infarction. In: Beeson P B, McDermott W, Wyngaarden J B, eds. Cecil Textbook of Medicine. 15th ed. Philadelphia: WB Saunders; 1979: 794
    Google Scholar
  • 11 Schwartz R B, Jones K M, Kalina P et al.. Hypertensive encephalopathy: findings on CT, MR imaging, and SPECT imaging in 14 cases.  AJR Am J Roentgenol. 1992;  159 (2) 379-383
    PubMedGoogle Scholar
  • 12 Bartynski W S. Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features.  AJNR Am J Neuroradiol. 2008;  29 (6) 1036-1042 Rev of imaging
    CrossrefPubMedGoogle Scholar
  • 13 Bartynski W S. Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema.  AJNR Am J Neuroradiol. 2008;  29 (6) 1043-1049
    CrossrefPubMedGoogle Scholar
  • 14 Chester E M, Agamanolis D P, Banker B Q, Victor M. Hypertensive encephalopathy: a clinicopathologic study of 20 cases.  Neurology. 1978;  28 (9 Pt 1) 928-939
    CrossrefPubMedGoogle Scholar
  • 15 Bartynski W S, Zeigler Z, Spearman M P, Lin L, Shadduck R K, Lister J. Etiology of cortical and white matter lesions in cyclosporin-A and FK-506 neurotoxicity.  AJNR Am J Neuroradiol. 2001;  22 (10) 1901-1914
    PubMedGoogle Scholar
  • 16 Wilson S E, de Groen P C, Aksamit A J, Wiesner R H, Garrity J A, Krom R A. Cyclosporin A-induced reversible cortical blindness.  J Clin Neuroophthalmol. 1988;  8 (4) 215-220
    PubMedGoogle Scholar
  • 17 MacKenzie E T, Strandgaard S, Graham D I, Jones J V, Harper A M, Farrar J K. Effects of acutely induced hypertension in cats on pial arteriolar caliber, local cerebral blood flow, and the blood-brain barrier.  Circ Res. 1976;  39 (1) 33-41
    PubMedGoogle Scholar
  • 18 Hansson H A, Johansson B, Blomstrand C. Ultrastructural studies on cerebrovascular permeability in acute hypertension.  Acta Neuropathol. 1975;  32 (3) 187-198
    CrossrefPubMedGoogle Scholar
  • 19 Apollon K M, Robinson J N, Schwartz R B, Norwitz E R. Cortical blindness in severe preeclampsia: computed tomography, magnetic resonance imaging, and single-photon-emission computed tomography findings.  Obstet Gynecol. 2000;  95 (6 Pt 2) 1017-1019
    CrossrefPubMedGoogle Scholar
  • 20 Johansson B, Li C L, Olsson Y, Klatzo I. The effect of acute arterial hypertension on the blood-brain barrier to protein tracers.  Acta Neuropathol. 1970;  16 (2) 117-124
    CrossrefPubMedGoogle Scholar
  • 21 Naidu K, Moodley J, Corr P, Hoffmann M. Single photon emission and cerebral computerised tomographic scan and transcranial Doppler sonographic findings in eclampsia.  Br J Obstet Gynaecol. 1997;  104 (10) 1165-1172
    PubMedGoogle Scholar
  • 22 Engelter S T, Petrella J R, Alberts M J, Provenzale J M. Assessment of cerebral microcirculation in a patient with hypertensive encephalopathy using MR perfusion imaging.  AJR Am J Roentgenol. 1999;  173 (6) 1491-1493
    PubMedGoogle Scholar
  • 23 Brubaker L M, Smith J K, Lee Y Z, Lin W, Castillo M. Hemodynamic and permeability changes in posterior reversible encephalopathy syndrome measured by dynamic susceptibility perfusion-weighted MR imaging.  AJNR Am J Neuroradiol. 2005;  26 (4) 825-830
    PubMedGoogle Scholar
  • 24 Zunker P, Ley-Pozo J, Louwen F, Schuierer G, Holzgreve W, Ringelstein E B. Cerebral hemodynamics in pre-eclampsia/eclampsia syndrome.  Ultrasound Obstet Gynecol. 1995;  6 (6) 411-415
    CrossrefPubMedGoogle Scholar
  • 25 Zunker P, Happe S, Georgiadis A L et al.. Maternal cerebral hemodynamics in pregnancy-related hypertension. A prospective transcranial Doppler study.  Ultrasound Obstet Gynecol. 2000;  16 (2) 179-187
    CrossrefPubMedGoogle Scholar
  • 26 Lassen N A. Cerebral blood flow and oxygen consumption in man.  Physiol Rev. 1959;  39 (2) 183-238
    PubMedGoogle Scholar
  • 27 Dethloff T J, Knudsen G M, Larsen F S. Cerebral blood flow autoregulation in experimental liver failure.  J Cereb Blood Flow Metab. 2008;  28 (5) 916-926
    CrossrefPubMedGoogle Scholar
  • 28 Lucas S J, Tzeng Y C, Galvin S D, Thomas K N, Ogoh S, Ainslie P N. Influence of changes in blood pressure on cerebral perfusion and oxygenation.  Hypertension. 2010;  55 (3) 698-705
    CrossrefPubMedGoogle Scholar
  • 29 Hernández M J, Brennan R W, Bowman G S. Autoregulation of cerebral blood flow in the newborn dog.  Brain Res. 1980;  184 (1) 199-202
    CrossrefPubMedGoogle Scholar
  • 30 Pasternak J F, Groothuis D R. Autoregulation of cerebral blood flow in the newborn beagle puppy.  Biol Neonate. 1985;  48 (2) 100-109
    PubMedGoogle Scholar
  • 31 Tyszczuk L, Meek J, Elwell C, Wyatt J S. Cerebral blood flow is independent of mean arterial blood pressure in preterm infants undergoing intensive care.  Pediatrics. 1998;  102 (2 Pt 1) 337-341
    CrossrefPubMedGoogle Scholar
  • 32 Pryds O, Edwards A D. Cerebral blood flow in the newborn infant.  Arch Dis Child Fetal Neonatal Ed. 1996;  74 (1) F63-F69
    CrossrefPubMedGoogle Scholar
  • 33 Schwartz R B, Feske S K, Polak J F et al.. Preeclampsia-eclampsia: clinical and neuroradiographic correlates and insights into the pathogenesis of hypertensive encephalopathy.  Radiology. 2000;  217 (2) 371-376
    PubMedGoogle Scholar
  • 34 Beausang-Linder M, Bill A. Cerebral circulation in acute arterial hypertension—protective effects of sympathetic nervous activity.  Acta Physiol Scand. 1981;  111 (2) 193-199
    CrossrefPubMedGoogle Scholar
  • 35 Olsson Y, Hossmann K A. Fine structural localization of exudated protein tracers in the brain.  Acta Neuropathol. 1970;  16 (2) 103-116
    CrossrefPubMedGoogle Scholar
  • 36 McCrae K R, Samuels P, Schreiber A D. Pregnancy-associated thrombocytopenia: pathogenesis and management.  Blood. 1992;  80 (11) 2697-2714
    PubMedGoogle Scholar
  • 37 Schwartz R B, Bravo S M, Klufas R A et al.. Cyclosporine neurotoxicity and its relationship to hypertensive encephalopathy: CT and MR findings in 16 cases.  AJR Am J Roentgenol. 1995;  165 (3) 627-631
    CrossrefPubMedGoogle Scholar
  • 38 Ay H, Buonanno F S, Schaefer P W et al.. Posterior leukoencephalopathy without severe hypertension: utility of diffusion-weighted MRI.  Neurology. 1998;  51 (5) 1369-1376
    CrossrefPubMedGoogle Scholar
  • 39 Wright R R, Mathews K D. Hypertensive encephalopathy in childhood.  J Child Neurol. 1996;  11 (3) 193-196
    CrossrefPubMedGoogle Scholar
  • 40 Bakshi R, Bates V E, Mechtler L L, Kinkel P R, Kinkel W R. Occipital lobe seizures as the major clinical manifestation of reversible posterior leukoencephalopathy syndrome: magnetic resonance imaging findings.  Epilepsia. 1998;  39 (3) 295-299
    CrossrefPubMedGoogle Scholar
  • 41 Gijtenbeek J M, van den Bent M J, Vecht C J. Cyclosporine neurotoxicity: a review.  J Neurol. 1999;  246 (5) 339-346
    CrossrefPubMedGoogle Scholar
  • 42 Feske S K, Sperling R A, Schwartz R B. Extensive reversible brain magnetic resonance lesions in a patient with HELLP syndrome.  J Neuroimaging. 1997;  7 (4) 247-250
    PubMedGoogle Scholar
  • 43 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 (4) 904-912
    CrossrefPubMedGoogle Scholar
  • 44 Covarrubias D J, Luetmer P H, Campeau N G. Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images.  AJNR Am J Neuroradiol. 2002;  23 (6) 1038-1048
    PubMedGoogle Scholar
  • 45 Schaefer P W, Buonanno F S, Gonzalez R G, Schwamm L H. Diffusion-weighted imaging discriminates between cytotoxic and vasogenic edema in a patient with eclampsia.  Stroke. 1997;  28 (5) 1082-1085
    CrossrefPubMedGoogle Scholar
  • 46 Koch S, Rabinstein A, Falcone S, Forteza A. Diffusion-weighted imaging shows cytotoxic and vasogenic edema in eclampsia.  AJNR Am J Neuroradiol. 2001;  22 (6) 1068-1070
    PubMedGoogle Scholar
  • 47 Sengar A R, Gupta R K, Dhanuka A K, Roy R, Das K. MR imaging, MR angiography, and MR spectroscopy of the brain in eclampsia.  AJNR Am J Neuroradiol. 1997;  18 (8) 1485-1490
    PubMedGoogle Scholar
  • 48 Eichler F S, Wang P, Wityk R J, Beauchamp Jr N J, Barker P B. Diffuse metabolic abnormalities in reversible posterior leukoencephalopathy syndrome.  AJNR Am J Neuroradiol. 2002;  23 (5) 833-837
    PubMedGoogle Scholar
  • 49 Reece D E, Frei-Lahr D A, Shepherd J D et al.. Neurologic complications in allogeneic bone marrow transplant patients receiving cyclosporin.  Bone Marrow Transplant. 1991;  8 (5) 393-401
    PubMedGoogle Scholar
  • 50 Calhoun D A, Oparil S. Treatment of hypertensive crisis.  N Engl J Med. 1990;  323 (17) 1177-1183
    CrossrefPubMedGoogle Scholar
  • 51 Vaughan C J, Delanty N. Hypertensive emergencies.  Lancet. 2000;  356 (9227) 411-417
    CrossrefPubMedGoogle Scholar
  • 52 Blumenfeld J D, Laragh J H. Management of hypertensive crises: the scientific basis for treatment decisions.  Am J Hypertens. 2001;  14 (11 Pt 1) 1154-1167
    CrossrefPubMedGoogle Scholar
  • 53 Wilson D J, Wallin J D, Vlachakis N D et al.. Intravenous labetalol in the treatment of severe hypertension and hypertensive emergencies.  Am J Med. 1983;  75 (4A) 95-102
    CrossrefPubMedGoogle Scholar
  • 54 Oparil S, Aronson S, Deeb G M et al.. Fenoldopam: a new parenteral antihypertensive: consensus roundtable on the management of perioperative hypertension and hypertensive crises.  Am J Hypertens. 1999;  12 (7) 653-664
    CrossrefPubMedGoogle Scholar
  • 55 Shusterman N H, Elliott W J, White W B. Fenoldopam, but not nitroprusside, improves renal function in severely hypertensive patients with impaired renal function.  Am J Med. 1993;  95 (2) 161-168
    CrossrefPubMedGoogle Scholar
  • 56 Houston M. Hypertensive emergencies and urgencies: pathophysiology and clinical aspects.  Am Heart J. 1986;  111 (1) 205-210
    PubMedGoogle Scholar
  • 57 Eclampsia Trial Collaborative Group . Which anticonvulsant for women with eclampsia? Evidence from the Collaborative Eclampsia Trial.  Lancet. 1995;  345 (8963) 1455-1463
    PubMedGoogle Scholar
  • 58 Lucas M J, Leveno K J, Cunningham F G. A comparison of magnesium sulfate with phenytoin for the prevention of eclampsia.  N Engl J Med. 1995;  333 (4) 201-205
    CrossrefPubMedGoogle Scholar
  • 59 Altman D, Carroli G, Duley L Magpie Trial Collaboration Group et al. Do women with pre-eclampsia, and their babies, benefit from magnesium sulphate? The Magpie Trial: a randomised placebo-controlled trial.  Lancet. 2002;  359 (9321) 1877-1890
    CrossrefPubMedGoogle Scholar
  • 60 Esen F, Erdem T, Aktan D et al.. Effects of magnesium administration on brain edema and blood-brain barrier breakdown after experimental traumatic brain injury in rats.  J Neurosurg Anesthesiol. 2003;  15 (2) 119-125
    CrossrefPubMedGoogle Scholar
  • 61 Esen F, Erdem T, Aktan D et al.. Effect of magnesium sulfate administration on blood-brain barrier in a rat model of intraperitoneal sepsis: a randomized controlled experimental study.  Crit Care. 2005;  9 (1) R18-R23
    CrossrefPubMedGoogle Scholar
  • 62 Kaya M, Gulturk S, Elmas I et al.. The effects of magnesium sulfate on blood-brain barrier disruption caused by intracarotid injection of hyperosmolar mannitol in rats.  Life Sci. 2004;  76 (2) 201-212
    CrossrefPubMedGoogle Scholar
  • 63 Euser A G, Bullinger L, Cipolla M J. Magnesium sulphate treatment decreases blood-brain barrier permeability during acute hypertension in pregnant rats.  Exp Physiol. 2008;  93 (2) 254-261
    PubMedGoogle Scholar
  • 64 Lam A M, Visco E, Ludbrook G. Influence of magnesium on cerebral blood flow autoregulation and CO2 reactivity in humans.  [Abstract] Anesthesiology. 2002;  96 A262
    PubMedGoogle Scholar
  • 65 Sherman R, Armory P, Moody P, Hope T, Mahajan R P. Effects of magnesium sulphate on cerebral haemodynamics in healthy volunteers: a transcranial Doppler study.  Br J Anaesth. 2003;  91 (2) 273-275
    CrossrefPubMedGoogle Scholar
  • 66 James M F, Cronjé L. Pheochromocytoma crisis: the use of magnesium sulfate.  Anesth Analg. 2004;  99 (3) 680-686
    CrossrefPubMedGoogle Scholar
  • 67 James M FM. Magnesium: an emerging drug in anaesthesia.  Br J Anaesth. 2009;  103 (4) 465-467
    CrossrefPubMedGoogle Scholar
  • 68 O'Riordan J A. Pheochromocytomas and anesthesia.  Int Anesthesiol Clin. 1997;  35 (4) 99-127
    PubMedGoogle Scholar

Steven K FeskeM.D. 

Division of Stroke, Department of Neurology

Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115

Email: sfeske@partners.org

      >