Atypical brain stem variant of posterior reversible encephalopathy syndrome (PRES)
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1 Atypical brain stem variant of posterior reversible encephalopathy syndrome (PRES) Poster No.: C-2289 Congress: ECR 2011 Type: Scientific Paper Authors: M. Kim, S. J. Jeon, S.-S. Choi, C. J. Song, I. K. Yu, G. H Chung ; Iksan/KR, Deajeon/KR, Jeonju/KR Keywords: Neuroradiology brain, MR DOI: /ecr2011/C-2289 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 25
2 Purpose Posterior reversible encephalopathy syndrome(pres) is characterized by clinical symptoms associated with imaging features of the typical presence of bilateral and symmetric vasogenic edema in the parietal and occipital lobes. It's predisposing factors are preeclampsia, eclampsia, allogenic bone marrow or solid organ transplantation, autoimmune diseases, high dose chemotherapy and so on. Pathomechanism of PRES is still remained unclear, but two opposing hypotheses are commonly cited. First one is hyperperfusion and vasogenic edema of brain parenchyma caused by severe hypertension with failed autoregulation. In the opposite side, second hypothesis is hypoperfusion and vasogenic edema due to compensatory vasoconstriction in the condition with hypertension. Unlikely with typical distribution of PRES such as parietal and occipital lobes, atypical PRES predominantly involves frontal lobe, basal ganglia, thalamus, brain stem, subcortical white matter and so on. Sympathetic innervation of cerebral circulation confers a protective effect from increases in systemic blood pressure. Because posterior circulation has lesser sympathetic innervations than anterior circulation, vasogenic edema occurs first in a vertebrovasilar vascular distribution. Thus, lesions occur first in the occipital lobes. Then more severe condition, cerebellum, brain stem and medulla oblongata may sequentially involved. Atypical brain stem variant of PRES is relatively rare and there were only few related reports. So, there were not been fully established differences between the PRES with typical distribution and Brain stem variants. the purpose of study was to investigate what differences between typical PRES and variant type. Images for this section: Page 2 of 25
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8 Methods and Materials MR reports among three hospitals were reviewed to identify patients with PRES between March 2003 and September There were 44 patients, 20 male and 24 female, ranging in age from 8 to 74 years. Mean age was 40.6 years. 44 patients were divided into two groups according to lesion distribution whether involve brain stem. Group I was composed of 16 patients presented with brain stem involvement. Group II was composed of 28 patients presented typical distributed lesions without brain stem involvement. MR images were obtained with 1.5 and 3.0T MR units. All 44 patients were performed MRI. DWIs were obtained for 41 patients. Follow-up MR was obtained for 24 patients. Two experienced neuroradiologists assessed the imaging studies, and they itemized and tabulated the locations of lesions that show hyperintensity on T2 and FLAIR sequences, diffusion restriction on DWI, and hemorrhage on GRE. Also, interval changes were assessed on follow-up MR. We reviewed the clinical records of all patients and paid attention to predisposing factors of PRES. Comparison of means by Mann-Whitney U test and Crosstabulation analysis by Chi-square test were done. All Statistical analysis was calculated by SPSS software. Images for this section: Page 8 of 25
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13 Results Mean systolic BP was (range: ) in group I and (range: ) in group II. Systolic BP was higher in group II (p=0.024) but others were no significant difference between two groups; age, sex, predisposing factor, symptom, and prognosis. Follow MR was performed in 25 patients (group I n=14, group II n=11). 23 of these patients had reversibility of lesion on follow up (group I: 13/14, group II: 10/11). 7 patients had sequela. In group I, 3 patients had sequela (2: encephalomalacia, 1: diffuse brain atrophy) and in group II, 4 patients had sequela (3: encephalomalacia, 1: death). However, there was no significant difference in two groups. Except for death, 4 of 6 patients who had sequela on follow up image, they had hemorrhage or irreversibility of lesion. In comparison between Groups divided by presence of sequelae, More diffusion restrictions and hemorrhagic lesions were indicated in patients with sequelae. And more reversibility of lesion was indicated in patients without sequelae. These results showed statistically significance. Images for this section: Page 13 of 25
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23 Conclusion In conclusion, Systolic BP has an influence of involvement of brain stem but stem involvement has no influence on prognosis. It seems that factors affecting to prognosis are not distribution of lesion but reversibility, diffusion restriction, and hemorrhage. Images for this section: Fig. 1 Page 23 of 25
24 References 1. Chester EM, Agamanolis DP, Banker BQ, Victor M. Hypertensive encephalopathy: a clinicopathologic study of 20 cases. Neurology 1977;28: Healton EB, Brust JC, Feinfeld DA, Thomson GE. Hypertensive encephalopathy and the neurologic manifestation of malignant hypertension. Neurology 1982;32: Schwartz RB, Jones KM, Kalina P, et al. Hypertensive encephalopathy: findings on CT, MR imaging, and SPECT imaging in 14 cases. AJR Am J Roentgenol 1992;159: Weingarten K, Barbut D, Filippi C, Zimmerman RD. Acute hypertensive encephalopathy: findings on spin-echo and gradientecho MR imaging. AJR Am J Roentgenol 1994;162: Skinhoj E, Standgoard S. Pathogenesis of hypertensive encephalopathy. Lancet 1973; Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334: Kang BW, Bae YJ, Cheon WH, Park SP, Suh CK. Two cases of hypertensive brainstem encephalopathy. J Korean Neurol Assoc 2003;21: Thambisetty M, Biousse V, Newman NJ. Hypertensive brainstem encephalopathy: clinical and radiographic features. J Neurol Sci 2003;208: Cruz-Flores S, de Assis Aquino Gondim F, Leira EC. Brainstem involvement in hypertensive encephalopathy: clinical and radiological findings. Neurology 2004;62: de Seze J, Mastain M, Stojkovic T, Ferriby D, Pruvo JP, Destee A, et al. Unusual MR findings of the brainstem in arterial hypertension. Am J Neuroradiol 2000;21: Morello F, Marino A, Cigolini M, Cappellari F. Hypertensive brain stem encephalopathy: clinically silent massive edema of the pons. Neurol Sci 2001;22: Schwartz RB, Mulkern RV, Gudbjartsson H, Jolesz F. Diffusionweighted MR imaging in hypertensive encephalopathy: clues to pathogenesis. AJNR Am J Neuroradiol 1998;19: Chang GY, Keane JR. Hypertensive brain stem encephalopathy. AJNR Am J Neuroradiol 2000;21: Chang GY, Keane JR. Hypertensive brainstem encephalopathy: three cases presenting with severe brainstem edema. Neurology 1999;53: Kumai Y, Toyoda K, Fujii K, Ibayashi S. Hypertensive encephalopathy extending into the whole brainstem and deep structures. Hypertens Res 2002;25: Page 24 of 25
25 15. Beausang-Linder M, Bill A. Cerebral circulation in acute arterial hypertension: protective effects of sympathetic nervous activity. Acta Physiol Scand 1989;111: Personal Information Atypical Brain Stem Variant of Posterior Reversible Encephalopathy Syndrome (PRES) Moo Sang Kim, Se Jeong Jeon, See Sung Choi, Chang June Song, In Kyu Yu, Gyung Ho Chung 4 1 Department of Radiology, Wonkwang University Hospital, 344-2, Shinyong-dong, Iksan, Jeonbuk, Korea 2 Department of Radiology, Chungnam National University Hospital, Deajeon, Korea 3 Department of Radiology, Eulji University Hospital, Deajeon, Korea 4 Department of Radiology, Jeonbuk University Hospital, Jeonju, Korea Corresponding author: Se Jeong Jeon Wonkwang University Hospital 344-2, Shinyong-dong, Iksan, Jeonbuk, Republic of Korea Tel: ; Fax: bluejin_@hanmail.net Zip code: Page 25 of 25
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