Posterior reversible encephalopathy syndrome in infection, sepsis, and shock

Abstract

Background and purpose: The cause of "posterior reversible encephalopathy syndrome" (PRES) is not established. We recently encountered several patients who developed PRES in the setting of severe infection. In this study, we comprehensively reviewed the clinical and imaging features in a large cohort of patients who developed PRES, with particular attention to those with isolated infection, sepsis, or shock (I/S/S).

Methods: The clinical/imaging features of 106 patients who developed PRES were comprehensively evaluated. In 25 of these patients, PRES occurred in association with severe I/S/S separate from transplantation. The clinical/imaging features (computer tomography, MR imaging, and MR angiography [MRA]) of the patients with I/S/S were further evaluated, including organ/tissue/blood culture results, mean arterial blood pressure (MAP) at toxicity, extent of cerebral edema, and presence of vasospasm.

Results: PRES occurred in association with I/S/S in 25 of 106 patients (23.6%), in addition to 4 other major clinical settings, including cyclosporine/FK-506 (post-transplant) neurotoxicity (46.2%), autoimmune disease (10.4%), postchemotherapy (3.7%), and eclampsia (10.4%). In the 25 patients with I/S/S, available cultures demonstrated a predominance of gram-positive organisms (84%). Blood pressure was "normal" at toxicity in 10 patients (MAP, 95 mm Hg); "severe" hypertension was present in 15 patients (MAP, 137 mm Hg). Extent of brain edema graded on imaging studies was greater in the normal MAP group compared with the severe hypertension group (P < .05). MRA demonstrated vasospasm in patients with severe hypertension and vessel "pruning" in the normal MAP group.

Conclusion: Infection/sepsis/shock may be an important cause of PRES, particularly in relation to infection with gram-positive organisms.

Figures

Fig 1.

Patient 1 is a 39-year-old man with baseline blood pressure 122/61 mm Hg who had severe pneumonia with bronchial obstruction. Bronchial lavage grew Staphylococcus aureus and blood culture grew coagulase-negative staphylococci. Neurotoxicity developed 13 days after positive cultures with severe headache followed by a seizure with blood pressure at toxicity 118/70 mm Hg. A–B, Brain MR imaging (FLAIR sequence) demonstrates moderate signal intensity abnormality from vasogenic edema in the occipital lobes bilaterally (open arrows) typical of the PRES pattern with full extension to the ventricular surface and moderate local cortical mass effect judged grade 3. Follow up imaging was not obtained, but the patient’s symptoms resolved completely.

Fig 2.

Patient 7 is a 68-year-old woman with necrotic pancreatitis, a pancreatic abscess, and baseline blood pressure of 141/67 mm Hg. Abscess grew mixed flora with coagulase-negative staphylococci and Acinetobacter baumannii and blood culture was positive for coagulase-negative staphylococci. Altered mental status with PRES developed 7 days after positive cultures with blood pressure at toxicity of 168/68 mm Hg. A-B, Brain MR imaging (FLAIR sequence) demonstrates vasogenic edema in the occipital (open arrows) and parietal region (curved arrows) bilaterally typical of PRES with extension into the deep white matter but no extension to the ventricle surface judged grade 2. C-D, Brain MR imaging (FLAIR sequence) obtained 1 month after initial imaging and toxicity demonstrates near complete resolution of the edema in the occipital (open arrows) and left parietal region (curved arrow) with complete resolution in the right parietal area (arrow).

Fig 3.

Patient 4 is a 56-year-old woman with a baseline blood pressure of 156/68 who developed a thigh abscess with culture growing mixed flora (Klebsiella pneumonial and enterococci). She developed MOD (coagulopathy, acute respiratory distress syndrome, acute renal failure, liver failure, and shock liver). On day 27 of intensive treatment of her infection and multiorgan failure, the patient developed altered mentation followed by a generalized seizure and blood pressure of 164/75 mm Hg. A-D, Brain MR imaging (FLAIR sequence) obtained the 1-day after neurotoxicity demonstrates severe and extensive vasogenic edema primarily involving the subcortical white matter of the parietal (curved arrows), occipital (open arrows), and temporal lobe regions (arrowheads) bilaterally with ventricular distortion from edema judged grade 5.

Fig 4.

Patient 8 is a 55-year-old woman with multiple liver metastases from renal cell carcinoma who underwent liver wedge resection and intraoperative chemotherapy infusion. Baseline blood pressure was 115/70 mm Hg. She developed ARDS and Streptococcus pneumoniae sepsis 3 days after resection followed by pneumonia (S aureus) and line sepsis (coagulase-negative staphylococci). Patient developed altered mental status 3 days after pneumonia and bacteremia with blood pressure at toxicity of 150/80 mm Hg. A-C, Brain CT images obtained at toxicity demonstrate vasogenic extensive edema in the occipital (open arrows), parietal (curved arrows), and frontal regions (arrows) bilaterally with focal edema also noted in the anterior limb internal capsule bilaterally (arrowheads) consistent with PRES with ventricular compression and deformity from the edema judged grade 5. D, Follow-up brain CT imaging performed 1 month after initial imaging demonstrates complete reversal of the PRES pattern shown here in the parietal (curved arrows) and frontal (arrows) regions bilaterally.

Fig 5.

Patient 2 was a 73-year-old woman who had undergone gastric surgery. Her baseline blood pressure of 157/77 mm Hg. She developed aspiration and pneumonia. Bronchial washings and blood cultures grew Pseudomonas aeruginosa. Neurotoxicity developed 6 days after positive cultures with altered mental status and blood pressure 128/80 mm Hg. A-C, Brain MR imaging (FLAIR sequence) obtained at the time of toxicity demonstrates an unusual pattern of vasogenic edema in the parietal region bilaterally much greater on the left (curved arrows) involving both cortex and some extension to the deep white matter judged grade 2. D, MRA obtained at the same time as imaging demonstrates diminutive severely “pruned” intracranial vessels, in particular MCA branches (arrows). E, Follow-up brain MR imaging (FLAIR sequence) obtained 1 month after initial imaging demonstrates complete resolution of the vasogenic edema bilaterally shown here only on the left (curved arrow). Incidental subdural hygromas are also present. F, Follow-up MRA also obtained 1 month after initial imaging demonstrates marked improvement in vessel visualization with partial reversal of the severe “pruning” and spasm in the MCA branches (arrows) bilaterally. The patient’s mental status completely normalized.

Fig 6.

Patient 19 was a 54-year-old woman who had undergone gastric bypass surgery. She developed a severe pneumonia 2 months after surgery that eventually required intubation along with antibiotic treatment. This occurred while she was being treated at an outside hospital. The patient developed vision changes, confusion, and hypertension (200/100 mm Hg) during treatment with initial CT imaging reported as negative, and she was transferred to our facility for advanced management. A-C, Brain MR imaging (FLAIR sequence) obtained 1 day after the development of toxicity and transfer demonstrated focal areas of vasogenic edema in the frontal lobes (arrows), parietal region (curved arrows), and occipital poles (open arrows) bilaterally with a mild degree of severity. Frontal lobe signal intensity is linear along the superior frontal sulcus (arrows), disconnected from the parietal abnormality (curved arrows) consistent with PRES and judged grade 1. D, MRA at the time of initial MR imaging demonstrates extensive vasospasm of first-, second-, and third-order branches in the anterior cerebral artery (arrowheads), middle cerebral artery (arrows), and posterior communicating artery (short arrows) vessels bilaterally. A “node”-like appearance is seen at many branch points of the main parent vessels typical of spasm (black arrows 4G). Similar findings were also present in the posterior circulation. E-F, Axial FLAIR image obtained on follow-up imaging study 11 days after the initial study demonstrates reversal of the vasogenic edema in all regions. G, Repeat MRA obtained 11 days after the initial study demonstrates resolution of the extensive vasospasm with a near-normal appearance of all vessels (arrows).