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Year : 2014  |  Volume : 7  |  Issue : 3  |  Page : 467-469

An atypical presentation of posterior reversible encephalopathy syndrome: diagnostic and therapeutic dilemmas

1 Department of Anaesthesiology, Gian Sagar Medical College and Hospital, Ram Nagar, Banur, Punjab, India
2 Department of Obstetrics and Gynaecology, Gian Sagar Medical College and Hospital, Ram Nagar, Banur, Punjab, India

Date of Submission14-Dec-2013
Date of Acceptance03-Aug-2014
Date of Web Publication27-Aug-2014

Correspondence Address:
Sukhminder J.S. Bajwa
Department of Anaesthesiology and Intensive Care, Gian Sagar Medical College and Hospital, Ram Nagar, Banur, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1687-7934.139599

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Anesthetic management in patients with altered sensorium presenting for emergency surgical procedures has always been a challenging task for the attending anesthesiologist. We report a 21-year-old primigravida who presented to us at term pregnancy with seizures, altered sensorium, and hypertension. A provisional diagnosis of eclampsia was made clinically, and the patient was taken up for emergency cesarean section under general anesthesia. She underwent uneventful operative delivery and was successfully extubated. However, during the postoperative period, she developed high-grade fever and gradually progressive respiratory and cardiovascular depression. She was shifted immediately to ICU after securing the airway and primary resuscitation. A possible suggestive differential diagnosis of posterior reversible encephalopathy syndrome was arrived, as postoperative computed tomography of the brain revealed bilateral hypodense cerebral hemispheres involving predominantly parieto-occipital lobes. Patient improved clinically with aggressive supportive treatment over the next 2 days and was successfully extubated. Diagnosis of posterior reversible encephalopathy syndrome is often delayed clinically in such circumstances and may lead to permanent neurological damage. An atypical presentation as in the present case may further add to the diagnostic dilemma and can possibly lead to institution of inappropriate therapeutic interventions. The present case emphasizes the importance of early recognition of the atypical cases and their timely management by the obstetricians, anesthesiologists, and the intensivists.

Keywords: eclampsia, hypertension, posterior reversible encephalopathy syndrome, pregnancy

How to cite this article:
Anand S, Bajwa SJ, Kaur J, Bajwa SK. An atypical presentation of posterior reversible encephalopathy syndrome: diagnostic and therapeutic dilemmas. Ain-Shams J Anaesthesiol 2014;7:467-9

How to cite this URL:
Anand S, Bajwa SJ, Kaur J, Bajwa SK. An atypical presentation of posterior reversible encephalopathy syndrome: diagnostic and therapeutic dilemmas. Ain-Shams J Anaesthesiol [serial online] 2014 [cited 2022 May 16];7:467-9. Available from:

  Introduction Top

Obstetrical emergencies can be highly unpredictable and can pose numerous clinical challenges to the attending obstetrician and the anesthesiologist [1]. Posterior reversible encephalopathy syndrome (PRES) is one such rare but well-recognized clinical and neuroradiological entity, which was first described in 1996 by Hinchey et al. [2]. The clinical symptomatology can be highly variable that can manifest as headache, nausea, encephalopathy, seizures, cortical visual disturbances, blindness, and coma [3,4]. PRES has been associated with numerous morbidities, including hypertensive encephalopathy, renal failure, autoimmune disorders such as systemic lupus erythomatosus, Henoch-Schφnlein purpura, thrombocytopenic syndrome, and immunosuppressive medications such as cyclosporine and neoplastic agents. It can also be associated with systemic infections, sepsis (gram-positive organisms predominate), and shock [5,6]. An increasing awareness of the clinical and radiographic presentation is required for timely diagnosis and prevention of short-term and long-term sequelae arising from PRES. We report a 21-year-old primigravida who presented to us at term pregnancy with seizures, altered sensorium, and hypertension, which was later diagnosed to be PRES but was successfully managed.

  Case report Top

A 21-year-old primigravida at 40 weeks' gestation presented in the emergency department with complaints of headache, altered sensorium, and seizure. There was no past history of seizures, hypertension, or any other neurological or cardiac disease. On examination, the patient was restless and disoriented with bilateral pedal edema. Pupils were equal in size and exhibited normal reaction to light. Her heart rate was 120 beats/min and blood pressure (BP) on admission was recorded, which was 170/110 and 172/108 mmHg, respectively, on two successive readings taken at an interval of 5 min. Her respiratory rate was 20-22 breaths/min displaying a pulse oximetry reading of 96% on room air. The systemic examination of other organ systems did not reveal anything significant and was absolutely normal. The hemogram, renal and liver function tests, and coagulation profile were also normal. However, ECG showed sinus tachycardia rhythm and urine examination revealed presence of albumin in traces. A provisional diagnosis of eclampsia was made and magnesium sulfate was initiated as per the Pritchard's regime. For effective control of hypertension, intravenous labetolol (20 mg) was given slowly. Considering the critical nature of the obstetric condition, the patient was shifted to the operation theater for emergency cesarean section, and general anesthesia was planned in light of critical clinical parameters. Rapid sequence induction was carried out using thiopentone (200 mg) and succinylcholine (50 mg), and the airway was secured with cuffed oral endotracheal tube of size 7 mm internal diameter. Anesthesia was maintained with isoflurane (0.5-1%) in oxygen and air (1 : 1), and atracurium (15 mg) was administered to facilitate muscular relaxation. A 2.2 kg male baby was delivered and was shifted to neonatal ICU because of low Apgar score of 7 and 8 at 1 and 5 min, respectively. Analgesia was achieved with 100 µg of fentanyl intraoperatively. Intraoperative systolic blood pressure remained between 130 and 150 mmHg, whereas diastolic blood pressure remained between 80 and 90 mmHg. Intraoperative blood loss was estimated at 500 ml and a total of 1 l of crystalloid solution (Ringer's lactate) was infused during the entire procedure, which lasted for 1 h. After the surgical procedure, residual muscular blockade was reversed with neostigmine (50 μg/kg) and glycopyrrolate (10 μg/kg), and patient was extubated on achieving fully awake state and establishment of rhythmic breathing with adequate tidal volume. For better monitoring of postoperative period, the patient was shifted to high dependency unit. Six hours after the cesarean section, the patient developed sudden hypotension with BP recording of 70/40 mmHg. After a rapid clinical review, a fluid challenge of 500 ml was given and dopamine infusion was also initiated at 5 μg/kg/min. Her temperature started increasing after the hypotensive episode and exhibited a maximum temperature of 41°C. During this period, the patient also became progressively drowsy and unresponsive. She was immediately intubated and shifted to ICU and was put on mechanical ventilation. Under all aseptic precautions, an arterial line was inserted and the right subclavian vein was cannulated, and fluids were infused to maintain a central venous pressure of 10-11 cmH 2 O. Paracetamol (1 g) administered intravenously and tepid sponging started to lower down the body temperature. Despite all these aggressive resuscitative efforts, hypotension persisted, which mandated the initiation of noradrenaline infusion in a titrated manner so as to achieve the primary target of minimum mean BP of 60 mmHg. Her BP and hemodynamic status stabilized almost after 12 h of this therapeutic intervention. Considering the seriousness of present neurological status, computed tomography (CT) scan was performed, which showed bilateral hypodense cerebral hemispheres involving predominantly parieto-occipital lobes suggestive of PRES [Figure 1]. Mannitol and frusemide were also added to the therapeutic armamentarium, and inotropes were gradually tapered off as the hemodynamic status and temperature got stabilized. On the second postoperative day, the patient's sensorium improved significantly. She was gradually weaned off from the ventilator and was successfully extubated. By the third postoperative day, the patient had completely recovered and did not exhibit any neurological sequelae and was shifted to the ward. Rest of her stay in the hospital was uneventful, and she was discharged on the seventh postoperative day.
Figure 1: Bilateral hypodense lesions involving gray and white matter of both cerebral hemispheres in the territory of the posterior circulation system.

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  Discussion Top

PRES is a clinicoradiological entity associated with diverse clinical disorders in which patients present with varied signs and symptoms such as alterations in mental status, headache, seizures, cortical visual disturbances including visual loss, occasional focal neurological signs, and rarely coma [7]. The primary pathophysiology is vasogenic edema predominantly in the parieto-occipital white matter, but there can be involvement of the brainstem, cerebellum, frontal lobes, and basal ganglia as well [3,7].

PRES, although rare, is presently a well-acknowledged clinical entity associated with pregnancy, pre-eclampsia, and eclampsia. The onset of PRES can occur from 28 weeks' gestational age [8] to day 13 postpartum [2].

The diagnosis is primarily based on clinical and radiological evidence. Currently, two schools of thoughts are considered important for the possible explanation of the pathogenesis of this syndrome. The first plausible explanation from literary evidence points to a disturbed arterial cerebrovascular autoregulation leading to hyperperfusion and breakdown of the blood-brain barrier resulting in vasogenic edema [9]. The second theory postulates that excessive arteriolar vasoconstriction results in decreased blood flow, ischemia, and consequent cytotoxic edema [10]. The posterior cerebral circulation is more commonly involved probably because of lower sympathetic innervations [11]. When the other areas of brain such as frontal, temporal lobes, cerebellum, thalamus, and brainstem are predominantly involved, the syndrome can be termed atypical [3]. Despite the marked neurological and radiological picture, the syndrome is reversible as evident from its nomenclature. However, a delayed diagnosis and treatment may lead to ischemia, cerebral infarction, and even death in some cases [12].

In the present case, although the patient presented with hypertension, seizures, and altered sensorium and was managed on the lines of eclampsia, there was an unanticipated and marked decrease in her BP during the postoperative period for which inotropes had to be initiated. She also developed high-grade fever with a single maximum spike of 41°C, which improved with antipyretics and cold sponging. Suspicion of septic shock was also made and noradrenaline was initiated along with dopamine depending upon the current clinical evidence. Postoperatively, her blood counts were normal, and urine and blood cultures were also sterile. CT scan however confirmed the diagnosis of PRES. MRI was not performed because of its nonavailability in our setup. Legriel et al. [13] suggested that CT scanning may be helpful in case of nonavailability of MRI.

Atypical presentations are common in PRES. Cardiovascular instability is also a known clinical association in PRES [14]. In the present case, timely supportive therapy and intervention resulted in successful management of this rare entity. As the patient showed complete and rapid clinical improvement, no follow-up scans were performed. However, the recognition of such cases is important for the obstetricians, intensive care physicians, and anesthesiologists.

  Acknowledgements Top

  References Top

1.Bajwa SK, Bajwa SJ. Delivering obstetrical critical care in developing nations. Int J Crit Illn Inj Sci 2012; 2:32-39.  Back to cited text no. 1
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2. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996; 334:494-500.  Back to cited text no. 2
3. Casey SO, Sampaio RC, Michel E, Truwit CL. Posterior reversible encephalopathy syndrome: utility of fluid-attenuation inversion recovery MR imaging in the detection of cortical and subcortical lesions. Am J Neuroradiol 2000; 21:1199-1206.  Back to cited text no. 3
4. Bartynski WS, Boardman JF. Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome. Am J Neuroradiol 2007; 28:1320-1327.  Back to cited text no. 4
5. Long TR, Hein BD, Brown MJ, Rydberg CH, Wass CT. Posterior reversible encephalopathy syndrome during pregnancy: seizures in a previously parturient. J Clin Anesth 2007; 19:145-148.  Back to cited text no. 5
6. Bartynski WS, Boardman JF, Zeigler ZR, Shadduck RK, Lister J. Posterior reversible encephalopathy syndrome in infection, sepsis and shock. Am J Neuroradiol 2006; 27:2179-2190.  Back to cited text no. 6
7. Puppala S, Hourihan. A pressing case of transient blindness. Br J Radiol 2005; 78:967-968.  Back to cited text no. 7
8. Servillo G, Apicella E, Striano P. Posterior reversible encephalopathy syndrome (PRES) in a parturient with preeclampsia after inadvertent dural puncture. Int J Obstet Anesth 2008; 17:88-89.  Back to cited text no. 8
9. Lamy C, Oppenheim C, Meder JF, Mas JL. Neuroimaging in posterior reversible encephalopathy syndrome. J Neuroimaging 2004; 14:89-96.  Back to cited text no. 9
10.Brubaker LM, Smith JK, Lee YZ, Lin W, Castillo M. Hemodynamics and permeability changes in posterior reversible encephalopathy syndrome measured by dynamic susceptibility perfusion weighted MR imaging. Am J Neuroradiol 2005; 26:825-830.  Back to cited text no. 10
11.Witlin AC, Friedman SA, Egerman RS, Frangieh AY, Sibai BM. Cerebrovascular disorders complicating pregnancy beyond eclampsia. Am J Obstet Gynecol 1997; 176:1139-1148.  Back to cited text no. 11
12.Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion weighted MR images. Am J Neuroradiol 2002; 23:1038-1048.  Back to cited text no. 12
13.Legriel S, Pico F, Azoulay E. Understanding posterior reversible encephalopathy syndrome. In: Vincet J-L, editor. Annual update in intensive care and emergency medicine. Berlin, Heidelberg: Springer; 2011. 631-653.  Back to cited text no. 13
14.Pratap JN, Down JF. Posterior reversible encephalopathy syndrome: a report of a case with atypical features. Anaesthesia 2008; 63:1245-1248.  Back to cited text no. 14


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