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Research ArticleBRAIN

Posterior Reversible Encephalopathy Syndrome in Infection, Sepsis, and Shock

W.S. Bartynski, J.F. Boardman, Z.R. Zeigler, R.K. Shadduck and J. Lister
American Journal of Neuroradiology November 2006, 27 (10) 2179-2190;
W.S. Bartynski
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J.F. Boardman
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Z.R. Zeigler
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R.K. Shadduck
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J. Lister
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  • Fig 1.
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    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.

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    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).

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    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.

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    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.

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    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.

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    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).

Tables

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    Table 1:

    Vasogenic edema scale

    Edema GradeDescriptionPoints
    Grade 1Cortex edema, limited subcortical white matter edema or both Involved regions nonconfluent Slight local cortex mass effect/distortion due to edema1
    Grade 2White matter edema slightly greater in thickness than cortex edema White matter edema extends into deep white matter Primarily without wide medial-to-lateral extension Some involved regions occasionally confluent Local cortical mass effect/distortion due to edema2
    Grade 3White matter edema substantially greater in thickness than cortex edema Some limited and focal extension to ventricular surface Developing wide medial-to-lateral extension Involved regions partially confluent Moderate local cortex mass effect/distortion due to the edema3
    Grade 4White matter edema substantially thicker than cortex edema Involved regions substantially extend to ventricular surface Diffuse wide medial-to-lateral extension Involved regions almost completely confluent No ventricular distortion due to the edema4
    Grade 5White matter edema severe and dominates cortex edema Involved regions fully confluent and continuous Diffuse wide medial-to-lateral extension Fully extends to the ventricular surface Ventricular deformity due to the edema5
    • View popup
    Table 2:

    Categories and timing of PRES neurotoxicity

    Toxicity CategoryNo. of PatientsSubgroupNo. of PatientsTime Point of PRES Relative to ‘Association’ Event
    0–1 mos (# pts)1–4 mos (# pts)4–12 mos (# pts)> 12 mos (# pts)Not Known (# pts)
    Cyclosporine/FK-506 toxicity49allo-BMT2616541
    SOT205357
    CsA only33
    Postchemotherapy4431
    Infection/sepsis/shock25Infection/Sepsis232111
    Hemorrhagic Shock22
    Eclampsia11Intrapartum33
    Delayed88
    Autoimmune111111
    Miscellaneous666
    Total10610656109823
    • Note:—# pts indicates number of patients; mos, months; SOT, solid organ transplant; CsA only, cyclosporine treatment in marrow diseases; PRES, posterior reversible encephalopathy syndrome; allo-BMT, allogenic bone marrow transplantation.

    • View popup
    Table 3:

    Clinical features of patients with infection, sepsis, and shock

    Pt #AgeSexMajor Clinical Problem and InfectionCultured OrganismS/SMODDelayPRES NeurotoxicityBP BaseBP ToxMAP Tox
    Organ/TissueBlood
    Group 1
        139MPneumonia with bronchial obstruction, decubitus ulcerS. aureusStaph coag negY13DHeadache Seizure122/61118/7086
        273FGastric surgery, pneumonia, aspirationPseudomonasP. aeruginosaYCR6DAltered MS157/77128/8078
        359FBowel perforation, abscess, sepsis, MOD, antiphospholipid syndromePseudomonasStaph coag negYCP13DAltered MS143/79150/5687
    Staph coag neg
        456FThigh abscessKlebsiellaYCP27DSeizure156/68164/75104
    Enterococcus F1LR
        524FCML in blast crisis, isolated bacteremia, sepsis, chemotherapyEnterobacterYPL20D (15D)Slurred speech122/82125/8598
    RAtaxia
    Seizure
        620MALL, chemotherapy, skin infectionStaph coag neg1. Diphtheroids–CP7DSeizure120/70150/88106
    2. Staph coag negLR
        768FNecrotic pancreatitis, pancreatic abscessStaph coag negStaph coag negYCP7DAltered MS141/67168/68101
    AcinetobacterL
        855FLiver metastatis, open hepatic chemotherapy infusion, pneumoniaS. aureus1. S. pneumoniaeYCP3DAltered MS115/70150/82104
    2. Staph coag negLR
        966FToe infection and abscess, diabeticS. aureusR9DSeizure132/52160/6093
    Staph coag neg
    Yeast
        1079FLap-cholecystectomy; severe abdominal hemorrhageYCP LR7DAltered MS138/73137/7495
    Group 2
        1161FPostoperative wound infectionS. aureusS. aureus11DHeadache145/65210/102138
    Vision loss
        1224FIsolated bacteremia 2 mo post Tx removalS. viridansR0DHeadache Seizure125/90170/126140
        1322MOriginal sickle cell crisis w/pneumonia-bacteremia, new sickle cell crisis w/heavy rectal colonizationN/AStaph coag negCR2MSickle crisis Seizure122/80200/100133
    Enterococcus F2
        1437MChronic septic arthritis, shoulder abscessS. aureusN/ACR?Headache Vision change175/85184/111135
    Seizure
        1526FCrohn disease, bowel perforation, abdominal abscessEnterococcus F1Staph coag negYCP7DSeizure115/65182/100126
    Enterococcus F2
    E coli
        1651FBreast carcinoma, auto-BMT, pneumoniaPseudomonas1. MicrococcusYC p L9DSeizures121/74164/100123
    Klebsiella2. Enterococcus
    Staph coag neg3. Staph coag neg
        1755MFoot infection, diabeticN/A on antibioticsC0DHeadache Vision change191/88215/115148
        1857FPost gastric stapling, acute UTIEnterococcus F1Staph coag neg2DAltered MS Seizure136/70203/93129
        1954FPneumonia, severe; intubatedN/A outside hosp9DVision change Altered MS112/85200/100133
        2081FAxillary abscess, chemotherapyS. aureus1. S. aureusYCL30DSlurred speech Altered MS130/52203/110141
    2. Staph coag negSeizure
        2137FNecrotizing pancreatitis, pneumoniaN/A outside hosp7DHeadache Seizure162/92220/126157
        2256FSickle cell crisis, pneumoniaS. viridansR4DAltered MS Seizure150/84170/110130
        2324FAlcohol intoxication, shock, bowel perforationN/A on antibioticsYCP LR6DSeizure140/90203/113143
        2438FPeritonitis, PD, renal insufficiencyPseudomonasPseudomonas14DAltered MS145/94210/110143
        2519FDialysis catheter hemorrhageYCP R15DHeadache Vision change149/86207/106140
    Seizure
    • Note:—S/S indicates clinically hemorrhagic shock or sepsis, severe sepsis, or septic shock before developing PRES; Y, yes; MOD; multiorgan dysfunction developed coincident with PRES; C, coagulation, drop in platelet count; P, pulmonary dysfunction-edema, intubation; L, hepatic dysfunction, rising and elevated bilirubin; R, renal dysfunction, rising and elevated creatinine; Delay, closest time from recognized infection/bacteremia/shock to onset of neurotoxicity and PRES; MS, mental status; BP, blood pressure (mm Hg); base, baseline; tox, at toxicity; MAP, mean arterial pressure; N/A, not available; Tx, transplant; PD, peritoneal dialysis; UTI, urinary tract infection; PRES, posterior reversible encephalopathy syndrome. CML, chronic myelogenous leukemia; ALL, acute lymphoblastic leukemia; auto-BMT, auto-bone marrow transplant; S aureus, Staphylococcus aureus; Staph coag neg, coagulase-negative Staphylococci; E coli, Escherichia coli; S viridans, Streptococci viridans; S pneumoniae, Streptococci pneumoniae. Multiple organisms listed together correspond to mixed flora infections. Numbered blood culture organisms correspond to separate blood culture results with different organisms in the PRES time frame. F1, faecalis; F2, faecium. In patient 5, initial neurotoxicity began 15 days after sepsis/7 days after chemotherapy. Initial imaging negative (16 days), repeat imaging demonstrating PRESS (20 days).

    • View popup
    Table 4:

    Imaging features in patients with infection, sepsis, and shock

    Pt #Imaging Locations of PRES Vasogenic EdemaMRAImaging ModalityDWIF/U ImagingClinical at DischargeEdema GradeMAP
    CTOPFMisc
    Group1
        1xxxMRn/oResolved386
        2Spasm-impMRNegRes278
        3xxxxMRNegResolvingResolved587
        4xxxxxtPruningMRn/oResolved5104
        5xxxxCTRes398
        6xCTExpired2106
        7PruningMRNegResolvingResolved2101
        8xxxxCTRes5104
        9xxxxNormalMRNegRes293
        10xxxMRNeg*ResolvingResolved485
    Group 2
        11xxxxt mbSpasm/prunMRNegNormal2138
        12xxxxMRNegRes3140
        13xxxxxbs mb cMRNegRes1133
        14xxxxxbsMRNegRes3135
        15xxxxCTResolved1126
        16xxxCTExpired2123
        17xxxtSpasmMRNegResolvingResolved2148
        18xxxxSpasmMRNegResolvingResolved3129
        19xxxSpasm-revMRNegRes1133
        20xxxxbsMRNegResolvingResolved2141
        21xxMRNeg—Resolved1157
        22xxxxSpasmMRNegRes4130
        23xxxxMRNegRes1143
        24xxxxSpasm-revMRNeg*Res*3143
        25xxxxMRn/oRes2140
    • Note:—PRES indicates posterior reversible encephalopathy syndrome; C, cerebellum; T, temporal lobe/inferior temporal-occipital junction; O, occipital lobe; P, parietal region; F, frontal lobes; bs, brain stem including medulla and pons; mb, midbrain; t, thalamus; c, caudate nucleus; rev, spasm reverses; imp, spasm improves; spasm/prun, middle crebral artery spasm with posterior cerebral artery pruning; DWI, diffusion-weighted image characteristics; n/o, DWI not obtained with MR imaging study; Neg, no evidence of restricted diffusion to suggest cytotoxic edema; Neg*, no restricted diffusion in the majority of vasogennic edema with focal region of restricted diffusion and hemorrhage noted; Res, vasogenic edema completely resolved on follow-up imaging studies; Res*, vasogenic edema resolved with minor persistent abnormal signal in region of previously noted restricted diffusion, infarction, or focal hemorrhage; Resolving, resolving vasogenic edema on follow-up imaging studies or resolving clinical symptoms related to PRES; Resolved, clinical symptoms at PRES neurotoxicity resolved; MAP, mean arterial pressure.

    • View popup
    Table 5:

    Vasogenic edema grade in Group 1 (Normotensive) and Group 2 (Severely Hypertensive) Patients

    Blood Pressure GroupVasogenic Edema Grade (Number of Patients)Group Average
    Grade 1Grade 2Grade 3Grade 4Grade 5
    Group 1042133.3
    Group 2554102.07
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W.S. Bartynski, J.F. Boardman, Z.R. Zeigler, R.K. Shadduck, J. Lister
Posterior Reversible Encephalopathy Syndrome in Infection, Sepsis, and Shock
American Journal of Neuroradiology Nov 2006, 27 (10) 2179-2190;

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Posterior Reversible Encephalopathy Syndrome in Infection, Sepsis, and Shock
W.S. Bartynski, J.F. Boardman, Z.R. Zeigler, R.K. Shadduck, J. Lister
American Journal of Neuroradiology Nov 2006, 27 (10) 2179-2190;
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