Posterior reversible encephalopathy syndrome
Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state that occurs secondary to the inability of posterior circulation to autoregulate in response to acute changes in blood pressure. Hyperperfusion with resultant disruption of the blood brain barrier results in vasogenic oedema, but not infarction, most commonly in the parieto-occipital regions.
PRES is also known as hypertensive encephalopathy or reversible posterior leukoencephalopathy.
The term PRES can be a misnomer as the syndrome can involve or extend beyond the posterior cerebrum. Furthermore, although most cases involve a resolution of changes with treatment of the precipitating cause and clinical recovery some patients can progress to develop permanent cerebral injury and be left with residual neurological defects.
It should not be confused with chronic hypertensive encephalopathy, also known as hypertensive microangiopathy, which results in microhemorrhages in the basal ganglia, pons and cerebellum.
Patients present with headache, seizures, encephalopathy and/or visual disturbance.
The syndrome can be precipitated by various clinical settings. The mechanism is not well understood but is thought to be related to altered integrity of the blood brain barrier. Two main theories have been proposed:
- high blood pressure: leads to loss of self-regulation, hyperperfusion with endothelial damage and vasogenic oedema
endothelial dysfunction: leads to vasoconstriction and hypoperfusion resulting in cerebral ischaemia and subsequent vasogenic oedema
Hypertension is not present or does not reach the upper limits to self-regulation (150-160 mmHg) in 25% of patients.
- severe hypertension
- haemolytic uraemic syndrome (HUS)
- thrombocytopaenic thromboic purpura (TTP)
- systemic lupus erythematosus (SLE)
- drug toxicity
- use of L-asparaginase
- bone marrow or stem cell transplantation
Most commonly there is vasogenic oedema within the occipital and parietal regions (~95% of cases), perhaps relating to the posterior cerebral artery supply. The oedema is usually symmetrical. Despite being termed posterior, PRES can be found in a non posterior distribution, mainly in watershed areas, including within the frontal, inferior temporal, cerebellar and brainstem regions 2. Both cortical and subcortical locations are affected.
There are three main imaging patterns:
- holohemispheric at watershed zones
- superior frontal sulcus
- parieto-occipital dominance
Other uncommon patterns of PRES in <5% include: purely unilateral, or "central" (brainstem or basal ganglia lacking cortical or subcortical white matter involvement).
Parenchymal infarctions and haemorrhage are associated with PRES in respectively 10-25% and 15% of cases.
The affected regions, as outlined above, are hypoattenuating.
Signal characteristics of affected regions include:
- T1: hypo intense in affected regions
- T1 C+ (Gd): patchy variable enhancement. It can be seen in ~35% of patients, whether leptomeningeal or cortical pattern.
- T2: hyperintense in affected regions
- DWI: usually normal
- ADC: signal increased in affected regions due to increased diffusion
- GRE: may show hypointense signal in cases of haemorrhage
- SWI: may show microhemorrhages in up to 50%
General imaging differential considerations include:
- 1. Foocharoen C, Tiamkao S, Srinakarin J et-al. Reversible posterior leukoencephalopathy caused by azathioprine in systemic lupus erythematosus. J Med Assoc Thai. 2006;89 (7): 1029-32. Pubmed citation
- 2. Bartynski WS, Boardman JF. Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol. 2007;28 (7): 1320-7. doi:10.3174/ajnr.A0549 - Pubmed citation
- 3. Bartynski WS. Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features. AJNR Am J Neuroradiol. 2008;29 (6): 1036-42. doi:10.3174/ajnr.A0928 - Pubmed citation
- 4. Bartynski WS. Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol. 2008;29 (6): 1043-9. doi:10.3174/ajnr.A0929 - Pubmed citation
- 5. Bartynski WS, Tan HP, Boardman JF et-al. Posterior reversible encephalopathy syndrome after solid organ transplantation. AJNR Am J Neuroradiol. 2008;29 (5): 924-30. doi:10.3174/ajnr.A0960 - Pubmed citation
- 6. Fugate JE, Claassen DO, Cloft HJ et-al. Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin. Proc. 2010;85 (5): 427-32. doi:10.4065/mcp.2009.0590 - Free text at pubmed - Pubmed citation
- 7. McKinney AM, Short J, Truwit CL, McKinney ZJ, Kozak OS, SantaCruz KS, Teksam M. Posterior reversible encephalopathy syndrome: incidence of atypical regions of involvement and imaging findings. AJR Am J Roentgenol. 2007 Oct;189(4):904-12. www.ncbi.nlm.nih.gov/pubmed/17885064
- 8. McKinney AM, Sarikaya B, Gustafson C, Truwit CL. Detection of microhemorrhage in posterior reversible encephalopathy syndrome using susceptibility-weighted imaging. AJNR Am J Neuroradiol. 2012 May;33(5):896-903. http://www.ncbi.nlm.nih.gov/pubmed/22241378
- 9. McKinney AM, Jagadeesan BD, Truwit CL. Central-variant posterior reversible encephalopathy syndrome: brainstem or basal ganglia involvement lacking cortical or subcortical cerebral edema. AJR Am J Roentgenol. 2013 Sep;201(3):631-8. http://www.ncbi.nlm.nih.gov/pubmed/23971457
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Posterior reversible encephalopathy syndrome (PRES)||✓|
|Reversible posterior leukoencephalopathy syndrome||✓|
|Reversible posterior cerebral edema syndrome||✓|
|Acute hypertensive encephalopathy||✓|
|Reversible posterior cerebral oedema syndrome||✗|