|Year : 2016 | Volume
| Issue : 1 | Page : 17-20
Posterior reversible encephalopathy syndrome: An atypical postpartum complication
Debashish Paul1, Sachin Narayan Kulkarni1, Mili Das Choudhury2, GD Maity2
1 Department of Anaesthesiology, Indian Naval Hospital Kalyani, Visakhapatnam, Andhra Pradesh, India
2 Department of Obstetrics and Gynaecology, Indian Naval Hospital Kalyani, Visakhapatnam, Andhra Pradesh, India
|Date of Submission||29-Nov-2015|
|Date of Acceptance||17-Mar-2016|
|Date of Web Publication||17-Jun-2016|
Department of Anaesthesiology and Critical Care, Indian Naval Hospital, Kalyani, Vishakhapatnam - 530 005, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Posterior reversible encephalopathy syndrome (PRES) is presented by headache, altered mental status, blurring of vision, vomiting and seizure in conjunction with radiological finding of posterior cerebral white matter edema. Data suggest that most cases occur in young middle-aged with marked female preponderance, hypertension being the most common cause. In this case, it was diagnosed in a normotensive patient in the postnatal period that underwent cesarean section. The initial symptoms had misled toward a diagnosis of postdural puncture headache. Symptomatic treatment was started immediately in the ICU. This is an interesting case as the patient was a normotensive one without any other contributory factors and there was unanticipated delay in diagnosing the case until the time we could get a magnetic resonance imaging report.
Keywords: Postdural puncture headache, posterior cerebral white matter edema, posterior reversible encephalopathy syndrome, seizure and hypertension in pregnancy
|How to cite this article:|
Paul D, Kulkarni SN, Choudhury MD, Maity G D. Posterior reversible encephalopathy syndrome: An atypical postpartum complication. Indian Anaesth Forum 2016;17:17-20
|How to cite this URL:|
Paul D, Kulkarni SN, Choudhury MD, Maity G D. Posterior reversible encephalopathy syndrome: An atypical postpartum complication. Indian Anaesth Forum [serial online] 2016 [cited 2017 Mar 28];17:17-20. Available from: http://www.theiaforum.org/text.asp?2016/17/1/17/183579
| Introduction|| |
Posterior reversible encephalopathy syndrome (PRES) is a clinical, neuroradiological diagnosis presented by headache, altered mental status, blurring of vision, vomiting and seizure in conjunction with radiological finding of posterior cerebral white matter edema. Incidence of PRES is unknown, epidemiological data between 1988 and 2008 suggesting that most cases occur in young middle-aged are marked female predominance. Most common cause of PRES is hypertension followed by factors in the setting of a significant “systemic process,” include preeclampsia, transplantation (allogeneic bone marrow transplantation, organ transplantation), infection/sepsis/shock, autoimmune disease, and cancer chemotherapy. Vasogenic edema due to impaired cerebral autoregulation in hypertensive patients is the pathophysiology whereas immune response to other stimuli in nonhypertensive patient is the possible explanation. Although it is a reversible condition, PRES must be diagnosed early. In our case, initial symptoms had misled toward a diagnosis of postdural puncture headache (PDPH) until we could correlate magnetic resonance imaging (MRI) brain report to the clinical presentation and investigate to rule out other factors.
| Case Report|| |
A 28-year-old multigravida at 37 weeks underwent cesarean section under spinal anesthesia. The patient had no comorbidity and was normotensive, euglycemic with normal hematological and biochemical parameters. Subarachnoid block was administered with 26-gauge Quincke needle in the left lateral position, median approach. There was no bloody tap and the block was given in single attempt. Intraoperative period was uneventful. On the 3rd postoperative day, the patient complained of neck pain and headache on standing and reduced pain while lying for 15–20 min. Analgesics, nonsteroidal anti-inflammatory drugs, hydration, and abdominal binder were advised suspecting it as a case of PDPH. There was no feature like neck stiffness on day 3. On day 4, intensity of pain increases which was even worse on day 5 with nausea and vomiting. Hydrocortisone and sumatriptan were added in the line of management of PDPH. Following development of generalized seizure on the same day, patient was shifted to the Intensive Care Unit (ICU) and intensive care was started for seizure with symptomatic treatment and monitoring of blood pressure and glycemic status. On day 6, the patient complained of blurring of vision. Clinical evaluation and examination reveal normal fundus with absent Kernig's sign. Cerebrospinal fluid (CSF) routine bacterial, fungal, and viral cultures were negative with predominant lymphocytes and increased globulin. Computed tomography (CT) of the brain excluded any intracranial bleeding or mass. MRI of the brain shows diffuse hyperintense signal in deep white matter and subcortical white matter in bilateral frontal and occipital regions of fluid-attenuated inversion recovery (FLAIR) sequence [Figure 1]. This finding of leptomeningeal enhancement and high-signal-intensity lesions in the subcortical white matter involving both hemispheres is consistent with PRES. The patient was treated accordingly. Seizure was controlled by lorazepam and was started on levetiracetam 500 mg bid. The patient did not require support for the maintenance of airway. From the 8th postoperative day onward, patient started improving gradually and subsequently shifted back to the ward with continuation of antiepileptic drugs. Repeat brain MRI-T2/FLAIR after 1 month showed no subcortical or cortical T2-hyperintensity in the occipital and frontal region [Figure 2]. Anti-epileptic drugs were tapered off with regular follow-up of the patient.
|Figure 1: (a) T2-weighted axial image shows diffuse hyperintense signal in deep white matter and subcortical white matter in bilateral frontal and occipital regions. (b) Coronal fluid-attenuated inversion recovery image shows diffuse hyperintense signal in deep white matter and subcortical white matter along the frontal and occipital regions|
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|Figure 2: Magnetic resonance imaging brain of the patient during follow-up in the outdoor|
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| Discussion|| |
Generalized seizure is the most common manifestation, but case reports of focal seizure in PRES are available. Visual changes range from blurring of vision to cortical blindness. Depending on the severity and the latency in initiating proper treatment, symptoms develop over hours and can persist for weeks. Acute hypertension, eclampsia are the most common causes of PRES. In this case, there was no evidence for the known factors. Blood pressure on day 4 was noted maximum as 140/96 mm of Hg. Cases of PRES with normal-low blood pressure values have been reported. Rapid elevation of blood pressure is the key factor rather than higher blood pressure. It is thought that by virtue of direct effect of pregnancy on perivascular innervations, particularly in pial vessels by mechanisms not yet elucidated can predispose to brain edema formation. Further, raised endogenous anti-angiogenic factors in pregnancy may play a role. In this case, initial clinical features in the postpartum period were confusing and the management was started considering it to be PDPH till the onset of seizure and associated symptoms. ICU admission was a good decision as 35–40% of patients with PRES required mechanical ventilation., Requirement of upper airway protection should be evaluated in unconscious patients or patients with seizure activity. CSF analysis is not required in PRES except for differential diagnosis. CT findings are often normal or nonspecific. Cerebral MRI is the key investigation in PRES. Proton density and T2-weighted images show regions of high signal indicating edema. FLAIR sequences improve the diagnosis of PRES and the detection of subcortical and cortical lesions. Differential diagnosis of PRES is challenging because of nonspecific clinical manifestations and multiplicity of radiological patterns. Several vascularly mediated cerebral entities with reversible changes seen in MRI and CT changes. Such entities are malignant hypertension, preeclampsia and eclampsia, cyclosporine toxicity, uremia and glomerulonephritis, hemolytic uremic syndrome, thrombotic and thrombocytopenic purpura. The differential diagnosis is related to the presence or absence of gray matter involvement. If both white and cerebral cortical gray matters are involved, infarction and infectious encephalitides are the primary differential consideration. If cerebral cortical gray matter is spared, possible differential diagnosis is likely multiple sclerosis, acute disseminated encephalomyelitis, progressive multifocal leukoencephalopathy, thrombosis of superior sagittal sinus and central pontine myelinolysis. In our case, MRI findings show edema in the white matter of posterior portion of the brain, especially in the occipital and parietal areas but different distributions have been reported. The imaging findings and clinical features of postpartum eclampsia are identical to those of hypertensive encephalopathy. Sudden elevations in systemic blood pressure exceed the autoregulatory capability of the brain vasculature. Regions of vasodilatation and vasoconstriction develop, especially in arterial boundary zones and there is breakdown of the blood–brain barrier with focal transudation of fluid and petechial hemorrhages. The pathologic process is also characterized by cerebral edema and petechial hemorrhages, especially in the parieto-occipital and occipital lobes. In our case, the repeat brain MRI-T2/FLAIR after resolution of symptoms has shown normal findings. Cases have been reported where follow-up scans have shown partial resolution. As per literature, resolution was noted within 8 days to 17 months after the first abnormal results. In this case, radiological finding was irrefutable. It is essential to treat PRES early once diagnosed. Symptomatic treatment with intensive care mainly for hypertensive emergencies and seizure disorder is required. Removal of causative agent is the need of the situation like toxins. In this case, we could not find out any particular drug as the cause. Interesting fact is that intravenous caffeine is one of the causative agents. Caffeine is recommended for management of PDPH. Though we did not administer intravenous caffeine in this case but we could have as we were thinking to manage PDPH before getting the radiological evidence of PRES and that might worsen the symptoms. If intubation is required in patients of PRES, then propofol or thiopental is a good choice since they have anticonvulsant effects. To conclude, this case exposes us to an unusual presentation of PRES in the initial period leading to wrong diagnosis until the classical symptoms and MRI findings became conclusive and patient was taken care of before significant delay. MRI finding was crucial in this case. It is therefore important for clinicians to consider PRES in all patients with neurological complaints in such scenario. Early diagnosis and resolution of underlying cause with generalized measure are the keystones of management.
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[Figure 1], [Figure 2]