Post-partum posterior reversible encephalopathy syndrome

B. V. Triveni; Salman Mohammed Sheikh; Deepak Shedde

doi:10.4103/2249-4472.132831

CASE REPORT

Year : 2014 | Volume : 4 | Issue : 1 | Page : 53-56

Post-partum posterior reversible encephalopathy syndrome

B. V. Triveni¹, Salman Mohammed Sheikh¹, Deepak Shedde²
¹ Department of Anaesthesiology , Yenepoya Medical College, Mangalore, Karnataka, India
² Department of Obstetrics and Gynaecology, Yenepoya Medical College, Mangalore, Karnataka, India

Date of Web Publication

20-May-2014

Correspondence Address:
B. V. Triveni
Department of Anaesthesiology, Yenepoya Medical College, Mangalore - 575 018, Karnataka
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2249-4472.132831

Abstract

Posterior Reversible Encephalopathy Syndrome (PRES) is a clinicopathological syndrome associated with various clinical conditions presenting with headache, encephalopathy, seizure and cortical visual disturbances. Radiological findings in PRES are thought to be due to vasogenic edema predominantly in posterior cerebral hemispheres and are reversible with appropriate management.
We present a case of post partum PRES,A 29 year old primigravida of 33 weeks 3 days period of gestation who presented to our hospital with painless bleeding per vagina and breathlessness. A provisional diagnosis of Ante partum Haemorrhage due to Marginal Placenta Previa was made and she was admitted for safe confinement. Caesarean section was performed for APH under subarachnoid block which was uneventful. On the fourth post operative day patient developed headache and generalised tonic clonic seizures. The provisional diagnosis of Postpartum PRES was made and confirmed with MRI. All other causes of postpartum seizures were ruled out. Patient was successfully treated with anticonvulsants, corticosteroids and supportive treatment
Postpartum PRES is a rare clinical condition mostly associated with hypertension, Preeclampsia and vasculitis .Early recognition and treatment can lead to complete recovery of the condition

Keywords: encephalopathy, headache, seizure

How to cite this article:
Triveni BV, Sheikh SM, Shedde D. Post-partum posterior reversible encephalopathy syndrome. J Obstet Anaesth Crit Care 2014;4:53-6

How to cite this URL:
Triveni BV, Sheikh SM, Shedde D. Post-partum posterior reversible encephalopathy syndrome. J Obstet Anaesth Crit Care [serial online] 2014 [cited 2023 Mar 31];4:53-6. Available from: https://www.joacc.com/text.asp?2014/4/1/53/132831

Introduction

Posterior reversible encephalopathy syndrome (PRES) is a clinicopathological syndrome associated with various clinical conditions presenting with headache, encephalopathy, seizure and cortical visual disturbances. ^[1],[2] Radiological findings in PRES are thought to be due to vasogenic edema predominantly in posterior cerebral hemispheres and are reversible with appropriate management. Many causes of PRES have been reported, which includes hypertensive encephalopathy, pre-eclampsia, eclampsia, renal failure, immunosuppressants such as cyclosporine and tacrolimus, acute intermittent porphyria, thrombotic thrombocytopenic purpura and systemic lupus erythematosus. ^[2],[3] Various cases of PRES developed during peri-partum have been reported. ^{[2],[3],[4],[5]} We report a case of PRES in a post-partum patient who had antepartum hemorrhage due to marginal placenta previa and underwent cesarean section under spinal anesthesia.

Case report

The case we present here is about a 29-year-old primigravida (62 kg, 158 cm) was admitted to the labor room with a history of vaginal bleed of 1 day duration. She had completed 33 weeks of gestation without any symptoms suggestive of pre-eclampsia or hypertension. On examination, she was conscious, oriented and cooperative. Her pulse rate was104 beats/min and blood pressure 130/80 mmHg with respiratory rate of 25/min. Chest examination revealed basal crepitations and raised jugular venous pressure. She had bilateral calf tenderness and Homan's sign was positive. Chest radiograph was taken with abdominal binders and showed homogenous ground glass appearance suggestive of acute lung injury [Figure 1].

Figure 1: Chest radiograph showing homogenous ground glass appearance suggestive of acute lung injury

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An ultrasound revealed marginal placenta previa with a viable fetus corresponding to 33 weeks gestation. Doppler study of both lower limb venous systems was essentially normal with no evidence of deep vein thrombosis. The patient was admitted in the hospital for same confinement. On the 5 ^th day of confinement, patient had about severe bleeding per vagina (>500 ml) and hence was posted for emergency lower segment cesarean section under spinal anesthesia. Her blood pressure was 100/60 mmHg and pulse rate 100 beats/min.

When she arrived to the operation theater, she was anxious; her heart rate and blood pressure were 100 beats/min and 130/80 mmHg respectively. Intravenous ranitidine 50 mg and metoclopramide 10 mg were administered as premedication to protect against aspiration.

In the operation theater, standard American Society of Anesthesiologists monitoring was instituted and spinal anesthesia was performed with 27-gauge Quincke needle at L4-5, using a midline approach under strict aseptic precautions after preloading the patient with 1000 ml of ringer lactate (@20 ml/kg body weight). 10 mg 0.5% hyperbaric bupivacaine was administered through spinal route to achieve a T-4 sensory level for surgery, which proceeded uneventfully, with the patient exhibiting stable vital signs. The patient delivered single live baby (2140 g; Apgar score, 8-9) 5 min post-skin incision.

On the 4 ^th post-operative day, she developed headache and one episode of generalized tonic-clonic seizure. The seizure activity lasted for about 2 min and levetiracetam 1 g was injected immediately. The patient's blood pressure was 140/80 mmHg at the time of her seizure which was closely monitored. Patient was put on prophylactic doses of intravenous fosphenytoin and injection mannitol 100 mg 8 ^th hourly. Intravenous hydrocortisone 100 mg intravenously was given 8 ^th hourly. No neurological deficit or injuries were sustained, but patient was drowsy. Magnetic resonance imaging (MRI) brain with contrast was ordered. High signal intensity on T2-weighted image and increased fluid-attenuated inversion recovery (FLAIR) foci were seen in the bilateral frontal, parietal, occipital cortex, sub cortical white matter, Bilateral cerebellar hemispheres (right side more than left), caudal midbrain and pons corresponding with the MRI findings suggesting PRES [Figure 2] and [Figure 3]. Antinuclear antibodies (ANA) and antiphospholipid antibody-immunoglobulin M (APLA-IgM) profile were ordered.

Figure 2: Magnetic resonance imaging showing evidence of posterior reversible encephalopathy syndrome

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Figure 3: Magnetic resonance imaging showing evidence of posterior reversible encephalopathy syndrome

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Post-dural puncture headache (PDPH) because of diffuse nature of headache with no postural variation and as 27 gauge spinal needle used for spinal block was unlikely to cause PDPH. Other causes of headache were also ruled out as patient had no signs suggestive of meningitis such as nuchal rigidity. The negative ANA, anti-double stranded deoxyribonucleic acid and APLA-IgM report ruled out vasculitis. MRI ruled out any lesion suspicious of pituitary gland hemorrhage or pituitary apoplexy.

Over the next 6 h, patient continued to complain of mild headaches that responded moderately to oral paracetamol. On the 2 ^nd day after the seizure, the patient's visual acuity improved and no evidence of papilledema and her cognitive defects but headache persisted. Intravenous fosphenytoin, mannitol and corticosteroids were continued. On the 4 ^th day after the seizure, her blood pressure was 130/75 mmHg, headache resolved. The patient was observed for the next 5 days. A follow-up MRI of the brain was performed 9 days after the initial MRI. High signal intensity and increased FLAIR signal foci previously noted had completely resolved. The reversibility of the initial MRI findings helped to confirm the diagnosis of PRES. The patient was discharged on the 10 ^th post-operative day.

Discussion

The differential diagnosis for seizures in the late post-partum period includes eclampsia, subarachnoid hemorrhage, intracerebral hemorrhage, thrombotic phenomena, intracranial neoplasm, head trauma, idiopathic epilepsy, infection (meningoencephalitis), amniotic fluid embolism, post-partum angiopathy, arterial blood gas analysis revealed respiratory alkalosis [Table 1]. ^[6],[7] There was no past history of epilepsy or head injury post-dural puncture spinal headache usually presents within 24-48 h. Brain MRI ruled out intracranial bleed, ischemia secondary to thromboembolism, vasospasm or space occupying lesion. Amniotic fluid embolism rarely occurs after 48 h post-partum and generally presents with cardiopulmonary collapse and coagulopathy which was not seen in our patient. Possibility of systemic vasculitis was considered, but the ANA evaluation was negative. An alternative explanation is the possibility of post-partum angiopathy. This diagnosis should be considered in a post-partum patient with hypertension and headache but no proteinuria but patient in our case was normotensive. Post-partum angiopathy is a form of reversible cerebral segmental vasoconstriction characterized by severe "thunderclap" headaches, seizures, focal neurological deficits and segmental narrowing and dilatation of large and medium sized arteries. Typically, scanning reveals ischemic lesions but MRI findings consistent with reversible posterior leukoencephalopathy syndrome have been reported.

PRES is a multifactorial disease entity with majority of patients presenting hypertension but does occur rarely in patients with normal blood pressure with immune-tolerant conditions such as peri-partum or using immunosuppressants, undetected hypertension, and impaired transient cerebral autoregulation. ^[2],[8],[9]

Table 1: Arterial blood gas analysis at 98% SpO2 revealed respiratory alkalosis with CO2 washout

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The pathophysiology of PRES is still obscure. The physiological accumulation of fluid during pregnancy may increase the endothelial permeability of cerebral vasculatures, which further deteriorated ^[4],[5],[8] when blood pressure exceeds the threshold of the autoregulation center of the brain resulting in the vasogenic edema. The accompanying endothelial damage is reversible when hypertension is relieved which emphasizes the relationship between the severity of vasogenic edema and the severity of hypertension. ^[3],[8] Therefore, the control of hypertension in patients with PRES is of vital importance in reversing its pathophysiologic progress. ^[10],[11] Notably, there was only mild and transient hypertension in this case.

In this case, patient presented with vaginal bleed and diagnosis of marginal placenta previa was made. She had no history of hypertension in the past or in the antenatal period which rules out the possibility of pregnancy induced hypertension. No significant history suggestive of seizures or neurological symptoms were noted upon history. Possibility of other causes of headache was ruled out by history, clinical examination and MRI.

Within 5 days after the patient's tonic-clonic seizure, all neurologic symptoms and signs were relieved and blood pressure remained within adequate range without any medication, suggesting that the patient recovered from PRES without any sequelae.

MRI brain has been considered the most appropriate tool in diagnosing PRES and demonstrating changes in brain lesions. ^[3],[5] Usually, it involves the posterior occipito-parietal lobes and hence the nomenclature PRES. ^[9] In our case, it involved bilateral frontal, parietal, occipital cortex, subcortical white matter, bilateral cerebellar hemispheres (right side more than left), caudal midbrain and pons.

Early diagnosis and management of PRES is critical to avoid irreversible ischemic damage or death. ^{[2],[3],[12],[13]} Recognition at the earliest and prompt initiation of the supportive measures can prevent permanent neurologic damage and thereby the associated morbidity. Delayed diagnosis and therapy can result in permanent damage to affected brain tissue. The key to safe motherhood in such women is multidisciplinary care in the peri- and post-partum period.

Conclusion

This is a rare case of post-partum PRES which was diagnosed by exclusion.

References

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