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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 9  |  Issue : 2  |  Page : 246-249

Posterior reversible encephalopathy syndrome in pregnancy: A case series of 5 cases


1 Department of Obstetrics and Gynecology, MGM Medical College and Hospital, MGM Institute of Health Sciences, (Deemed to be University), Kamothe, Navi Mumbai -410209, Maharashtra, India
2 MGM Hospital, Sector -4E, Kalamboli, Navi Mumbai-410218, Maharashtra, India

Date of Submission08-Mar-2022
Date of Acceptance28-Apr-2022
Date of Web Publication17-Jun-2022

Correspondence Address:
Dr. Vaibhavi Birle
Department of Obstetrics and Gynecology, MGM Medical College and Hospital, MGM Institute of Health Sciences, (Deemed to be University), Kamothe, Navi Mumbai -410209, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mgmj.mgmj_30_22

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  Abstract 

Posterior reversible encephalopathy syndrome (PRES) is a rare clinic-radiological syndrome associated with acute changes in blood pressure during pregnancy. It is characterized symptomatically by headache, seizures, altered mental status, and visual blurring. Radiological changes are white matter vasogenic edema affecting the occipital and parietal lobes of the brain. It is being increasingly recognized due to increased institutional deliveries and advances in imaging particularly magnetic resonance imaging (MRI). Despite the increasing detection, the prediction of PRES and patient factors affecting susceptibility is still not clear. Hence, we conducted a retrospective study to analyze the factors associated with PRES at our tertiary care center.

Keywords: Eclampsia, posterior reversible encephalopathy syndrome, preeclampsia, pregnancy


How to cite this article:
Birle V, Dongargaonkar P, Sharma S, Gaiwal AS. Posterior reversible encephalopathy syndrome in pregnancy: A case series of 5 cases. MGM J Med Sci 2022;9:246-9

How to cite this URL:
Birle V, Dongargaonkar P, Sharma S, Gaiwal AS. Posterior reversible encephalopathy syndrome in pregnancy: A case series of 5 cases. MGM J Med Sci [serial online] 2022 [cited 2022 Aug 13];9:246-9. Available from: http://www.mgmjms.com/text.asp?2022/9/2/246/347690




  Introduction Top


Posterior reversible encephalopathy syndrome (PRES) was first described by Hinchey in 1996. It is characterized by subacute neurologic and radiographic findings. It is attributed to numerous factors but no definite causative etiology or precipitating factor. It is predominantly associated with malignant hypertension, eclampsia, and medical treatment as immunosuppressive therapy.[1],[2] The global incidence of PRES is unknown. Though it has been reported in patients of 4 to 90 years of age, most cases occur in young to middle-aged adults. A marked female preponderance is observed which may reflect some of the underlying causes. Clinically, PRES presents with headache, seizures, encephalopathy, visual disturbances, and focal neurological symptoms. As the name suggests, reversibility of these symptoms is one of the hallmarks of the disease.[3] Diagnosis of PRES is made based on history, clinical examination, and radiologic findings of symmetric bilateral hyper-intensities on T2-weighted magnetic resonance imaging (MRIs) representing vasogenic edema.[4] Management of PRES includes removal of any underlying cause, optimal reduction in blood pressure, and seizure management. Early diagnosis plays an important role in reducing morbidity and improving patient prognosis.[1] The current hypothesis proposes dysfunctional cerebral vascular auto-regulation leading to a cascade of arteriolar vasodilation, capillary damage, and subsequent cortical and subcortical vasogenic edema as a possible mechanism.[5],[6],[7],[8],[9],[10],[11] No clinical studies are available till now regarding patients with PRES needing life-sustaining treatments. The improved knowledge and research about factors influencing the outcome of PRES will result in better early management, less morbidity, and mortality. According to studies, delayed diagnosis and treatment may lead to mortality or irreversible neurological deficit.[12] In Eclampsia, preeclampsia, or PIH immediate control of blood pressure, anti-convulsive therapy, and temporary renal replacement therapy (hemodialysis/peritoneal dialysis) as required for the treatment of PRES.[12] In the case of posterior reversible encephalopathy syndrome in a patient with COVID-19 disease, possibly related to anti-IL-1 or anti-IL-6, suggests that anti-interleukin treatments may cause this syndrome, at least in patients with predisposing conditions such as infections and hydro electrolytic disorders.[13]

There are many hypotheses regarding the pathophysiology of PRES. The most accepted hypothesis is the vasospasm/hyperperfusion doctrine. This doctrine suggests the various risk factors cause vasospasm or vasoconstriction, followed by in turn causing brain hyperperfusion, and cerebral systemic immune response (toxemia of pregnancy), thereby leading to systemic endothelial cell activation and injury. Activated endothelial cells secrete large amounts of inflammatory mediators and vasoconstrictor substances, induce diffuse systemic vascular contraction, and then cause brain vasogenic edema.[14] Some studies have also reported calcineurin inhibitors to be linked with the development of PRES as attributed to the release of vasoconstrictive substances and also the aggravation of hypomagnesemia with arterial hypertension.[15],[16] But Hammerstrom and colleagues observed an average increase of 35% in the mean arterial blood pressure under a Tacrolimus regimen in a retrospective study.[17]


  Materials and methods Top


It is a retrospective study, therefore, approval of the Institutional Ethics Committee as well as the need for informed consent, have been waived off. The records of all obstetric patients were reviewed manually who were seeking care at MGM Medical College and Hospital, Navi Mumbai, India from January 1, 2020, to December 31, 2020, with the diagnosis of eclampsia. Of these patients, 5 had a confirmed diagnosis of eclampsia. based on the International Classification of Diseases, Ninth Revision codes, and the presence of previously published and widely accepted clinical criteria of hypertension, proteinuria, and seizure activity not attributable to other causes. After the diagnosis of eclampsia, all patients underwent magnetic resonance imaging (MRI) of the brain. All 5 patients had follow-up neuroimaging between 30 days after their initial studies as advised by a multidisciplinary team. The patterns of changes, as well as evidence of permanent neurologic abnormalities, were noted which are given in [Table 1].
Table 1: Shows the clinical and radiological findings of the patients

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  Results Top


In our study, the age of the patients varied from 18 to 26 years. As far as symptomology is concerned, 4 out of 5 patients complained of generalized headache, 3 patients had complaints of drowsiness and two patients complained of visual disturbances. All the patients had raised systolic and diastolic blood pressure. Four patients out of five were primigravida. MRI showed parieto-occipital changes in all patients, suggestive of PRES.

Maternal outcome

All the patients were admitted up to day 10 of delivery. None of the patients had any sensory or motor neurological deficit. The headache subsided in all patients within 2 days of seizures. All patients were followed up after 3 months with an MRI brain. MRI brain showed subsidence. Three of the patients had normal deliveries and two had undergone cesarean section. Both the cesarean sections were done for obstetric indications.

Neonatal outcome

1/5 iatrogenic preterm delivery was done given severe pre-eclampsia other 4 were term deliveries.


  Discussion Top


PRES syndrome is a clinical-radiological syndrome characterized symptomatically by headache, seizures, altered mental status, and visual disturbances with radiological changes of white matter (vasogenic edema) affecting the posterior occipital and parietal lobes of the brain. There is a wide range of ailments like severe infection, organ transplantation, sepsis, autoimmune diseases, pre-eclampsia, and eclampsia that could be responsible for PRES.[18] In our series of 5 cases, the only cause of PRES syndrome was eclampsia. During the duration of our study 32 eclampsia patients were admitted to the hospital. Based on the clinical examination of the patient, 5 patients were further investigated with MRI brain imaging and were diagnosed with PRES. As MRI is the only modality to diagnose PRES many of the cases remain undiagnosed.

In our study, the patients were of the age group of 18 years to 26 years with a mean age of 22 years. This was in agreement with another study where the mean age of the patients was of 25 years. In our study, four of the patients were primigravida. Other authors have also shown that PRES was mostly seen in primigravida patients. In our case series, all the patients with PRES syndrome reversed back to normal after a few days of appropriate management. However, some studies have shown permanent neurological damage secondary to cerebral infarction or hemorrhage and trans-tentorial herniation resulting in death, in few patients.

Our study result shows reversible PRES in mothers after proper line of management as other studies support our views and show reversible changes after a few days of treatment in the majority of cases.

Several clinical studies have indicated that eclamptic seizure activity may occur in patients with minimal elevations in blood pressure. This has fuelled the ongoing debate as to the role of hypertension, if any, in the pathogenesis of eclamptic seizures.[19],[20] It is possible that blood pressure alone is not the exclusive cause, and that endothelial dysfunction, which is a hallmark of preeclampsia, is also a contributing factor.[21]

Treatment for PRES is mainly aimed at blood pressure control, anti-seizure measures, and other supportive measures. Blood pressure should be managed with easily titratable medications like intravenous labetalol, nitroprusside, nicardipine, etc.[22] This may be one of the reasons that our patients did not suffer from any neurological sequelae contrary to some of the studies.[23],[24]

Three of our patients required intense care unit monitoring. It is similar to a study conducted by Lee VH et al.[25] that on average, about 40% of all patients diagnosed with PRES require intensive care monitoring and treatment due to severe complications such as status epilepticus, cerebral ischemia, intracerebral hemorrhage, or intracranial hypertension.


  Conclusion Top


There was no increase in maternal mortality among the patients of PRES however, there was an increase in hospital stay and maternal morbidity. Symptoms associated with the press were convulsion, headache, drowsiness, and some patients presented with the blurring of vision. One out of 5 patients had preterm deliveries. Follow-up MRI after 30 days was normal in all cases.

Ethical consideration

It is a retrospective study, therefore, informed patients’ consent has been waived off. There is no need to obtain Institutional Ethics Committee clearance because of being a retrospective study.

Financial support and sponsorship

Nil

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Granata G, Greco A, Iannella G, Granata M, Manno A, Savastano E, et al. Posterior reversible encephalopathy syndrome–insight into pathogenesis, clinical variants and treatment approaches. Autoimmun Rev 2015;14:830-6.  Back to cited text no. 1
    
2.
Legriel S, Pico F, Azoulay E Understanding posterior reversible encephalopathy syndrome. In: Vincent JL, editor. Annual Update in Intensive Care and Emergency Medicine 2011. Annual Update in Intensive Care and Emergency Medicine 2011. Berlin, Heidelberg: Springer; 2011. p. 631-53.  Back to cited text no. 2
    
3.
Ekawa Y, Shiota M, Tobiume T, Shimaoka M, Tsuritani M, Kotani Y, et al. Reversible posterior leukoencephalopathy syndrome accompanying eclampsia: Correct diagnosis using preoperative MRI. Tohoku J Exp Med 2012;226:55-8.  Back to cited text no. 3
    
4.
Singer S, Grommes C, Reiner AS, Rosenblum MK, DeAngelis LM Posterior reversible encephalopathy syndrome in patients with cancer. Oncologist 2015;20:806-11.  Back to cited text no. 4
    
5.
Cioffi P, Laudadio L, Nuzzo A, Belfiglio M, Petrelli F, Grappasonni I Gemcitabine-induced posterior reversible encephalopathy syndrome: A case report. J Oncol Pharm Pract 2012;18:299-302.  Back to cited text no. 5
    
6.
Rajasekhar A, George TJ Jr. Gemcitabine-induced reversible posterior leukoencephalopathy syndrome: A case report and review of the literature. Oncologist 2007;12:1332-5.  Back to cited text no. 6
    
7.
Maeda T, Kikuchi E, Matsumoto K, Yazawa S, Hagiuda J, Miyajima A, et al. Gemcitabine and cisplatin chemotherapy induced reversible posterior leukoencephalopathy syndrome in a bladder cancer patient. Int J Clin Oncol 2010;15:508-11.  Back to cited text no. 7
    
8.
Marrone LC, Marrone BF, de la Puerta Raya J, Gadonski G, da Costa JC Gemcitabine monotherapy associated with posterior reversible encephalopathy syndrome. Case Rep Oncol 2011;4:82-7.  Back to cited text no. 8
    
9.
Ito Y, Arahata Y, Goto Y, Hirayama M, Nagamutsu M, Yasuda T, et al. Cisplatin neurotoxicity presenting as reversible posterior leukoencephalopathy syndrome. AJNR Am J Neuroradiol 1998;19:415-7.  Back to cited text no. 9
    
10.
Kwon EJ, Kim SW, Kim KK, Seo HS, Kim DY A case of gemcitabine and cisplatin associated posterior reversible encephalopathy syndrome. Cancer Res Treat 2009;41:53-5.  Back to cited text no. 10
    
11.
Mitsuya K, Nakasu Y, Hayashi N, Yasui H, Ikeda T, Kuji S, et al. Posterior reversible encephalopathy syndrome associated with cancer therapy. No Shinkei Geka 2016;44:211-9.  Back to cited text no. 11
    
12.
Sudulagunta SR, Sodalagunta MB, Kumbhat M, Settikere Nataraju A Posterior reversible encephalopathy syndrome(PRES). Oxf Med Case Reports 2017;2017:omx011.  Back to cited text no. 12
    
13.
Llansó L, Urra X Posterior reversible encephalopathy syndrome in COVID-19 disease: A case-report. SN Compr Clin Med 2020;2:1900-2.  Back to cited text no. 13
    
14.
Zhang L, Wang Y, Shi L, Cao J, Li Z, Wáng YX Late postpartum eclampsia complicated with posterior reversible encephalopathy syndrome: A case report and a literature review. Quant Imaging Med Surg 2015;5:909-16.  Back to cited text no. 14
    
15.
Bechstein WO Neurotoxicity of calcineurin inhibitors: Impact and clinical management. Transpl Int 2000;13:313-26.  Back to cited text no. 15
    
16.
Gijtenbeek JM, van den Bent MJ, Vecht CJ Cyclosporine neurotoxicity: A review. J Neurol 1999;246:339-46.  Back to cited text no. 16
    
17.
Hammerstrom AE, Howell J, Gulbis A, Rondon G, Champlin RE, Popat U Tacrolimus-associated posterior reversible encephalopathy syndrome in hematopoietic allogeneic stem cell transplantation. Am J Hematol 2013;88:301-5.  Back to cited text no. 17
    
18.
Kastrup O, Gerwig M, Frings M, Diener HC Posterior reversible encephalopathy syndrome (PRES): Electroencephalographic findings and seizure patterns. J Neurol 2012;259:1383-9.  Back to cited text no. 18
    
19.
Fugate JE, Rabinstein AA Posterior reversible encephalopathy syndrome: Clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol 2015;14:914-25.  Back to cited text no. 19
    
20.
Marra A, Vargas M, Striano P, Del Guercio L, Buonanno P, Servillo G Posterior reversible encephalopathy syndrome: The endothelial hypotheses. Med Hypotheses 2014;82:619-22.  Back to cited text no. 20
    
21.
Wagner SJ, Acquah LA, Lindell EP, Craici IM, Wingo MT, Rose CH, et al. Posterior reversible encephalopathy syndrome and eclampsia: Pressing the case for more aggressive blood pressure control. Mayo Clin Proc 2011;86:851-6.  Back to cited text no. 21
    
22.
Machiraju PK, Alex NM, Safinaaz , Sankaran S Posterior reversible encephalopathy syndrome as the first manifestation of mixed connective tissue disorder: A case report. J Med Case Rep 2021;15:40.  Back to cited text no. 22
    
23.
Khan RB, Sadighi ZS, Zabrowski J, Gajjar A, Jeha S Imaging patterns and outcome of posterior reversible encephalopathy syndrome during childhood cancer treatment. Pediatr Blood Cancer 2016;63:523-6.  Back to cited text no. 23
    
24.
Heo K, Cho KH, Lee MK, Chung SJ, Cho YJ, Lee BI Development of epilepsy after posterior reversible encephalopathy syndrome. Seizure 2016;34:90-4.  Back to cited text no. 24
    
25.
Lee VH, Wijdicks EF, Manno EM, Rabinstein AA Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol 2008;65:205-10.  Back to cited text no. 25
    



 
 
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