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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 4  |  Issue : 2  |  Page : 109-111

Posterior reversible encephalopathy syndrome


1 Department of General Medicine, Pondicherry Institute of Medical Sciences, Puducherry, India
2 Department of Radiology, Pondicherry Institute of Medical Sciences, Puducherry, India

Date of Submission04-Oct-2018
Date of Acceptance15-Oct-2018
Date of Web Publication13-Dec-2018

Correspondence Address:
Nayyar Iqbal
Department of General Medicine, Pondicherry Institute of Medical Sciences, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrsm.jcrsm_36_18

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  Abstract 


Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiographical syndrome of varying etiologies. It is characterized by headache, confusion, seizures, visual loss, and raised blood pressure along with magnetic resonance imaging abnormalities. It is associated with a number of conditions such as hypertension, eclampsia, vasculitis, chemotherapeutic drugs, and postpartum state. We report the case of a 28-year-old female who developed PRES on her 8th day of postpartum along with subarachnoid hemorrhage.

Keywords: Eclampsia, hypertension, posterior reversible encephalopathy syndrome


How to cite this article:
Sivaguru N, Indira K M, Pradeep K, Gopinath L, Iqbal N. Posterior reversible encephalopathy syndrome. J Curr Res Sci Med 2018;4:109-11

How to cite this URL:
Sivaguru N, Indira K M, Pradeep K, Gopinath L, Iqbal N. Posterior reversible encephalopathy syndrome. J Curr Res Sci Med [serial online] 2018 [cited 2019 Jul 23];4:109-11. Available from: http://www.jcrsmed.org/text.asp?2018/4/2/109/247498




  Introduction Top


Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiographical syndrome of varying etiologies. It is also known as reversible leukoencephalopathy syndrome, reversible posterior cerebral edema syndrome, hyperperfusion encephalopathy, and brain capillary leak syndrome. PRES was first described by Hinchey et al. in the year 1996.[1] PRES is a syndrome characterized by headache, confusion, seizures, visual loss, and raised blood pressure (BP) coupled with magnetic resonance imaging (MRI) abnormalities. It occurs in association with a number of conditions such as hypertension, preeclampsia, eclampsia, postpartum state, organ transplantation, chemotherapeutic drugs, vasculitis-like systemic lupus erythematosus, polyarthritis nodosa, hemolytic uremic syndrome, and porphyria.[2] PRES has been reported in various case series, but incidence is not known. Patients of all age groups are susceptible to this condition. It has been reported in patients as young as 2 years old and as old as 90 years old.[3] Female are more prone to this condition. Hypertensive disorder and immunosuppressive therapies are risk factors for PRES.[1],[4] We report the case of a 28-year-old female who developed PRES on her 8th day of postpartum along with subarachnoid hemorrhage.


  Case Report Top


A 28-year-old female (Para 1, Live 1), on her 8th day of postpartum period, came to the casualty with the complaint of two episodes of generalized tonic clonic seizures, associated with tongue bite and loss of consciousness. She had a history of headache prior to the onset of seizures. She delivered a baby girl of 2.8 kg through normal vaginal delivery. Her antenatal period was uneventful, though she had hypothyroidism during pregnancy for which she took tablet thyroxine 25 mcg/day. There was no history of preeclampsia, eclampsia, pregnancy-induced hypertension, and gestational diabetes mellitus. Till day 7 of her postpartum period, her BP was within normal limits. There was no past history of acquired or congenital heart diseases and seizure disorder. On examination, she was afebrile and confused. Her general examination revealed BP of 150/90 mmHg and pulse of 90 beats/min. Central nervous system examination revealed early papilledema with no motor or sensory deficits. Other systemic examination was normal. Her laboratory investigations were within normal limits including her autoimmune antibodies. She was nonreactive for HIV, hepatitis B virus surface antigen, and anti-hepatitis C virus antibodies. Her urine routine examination was negative for proteinuria. Her MRI of brain showed features suggestive of bilateral occipital hyperintensity suggestive of postpartum PRES and subarachnoid hemorrhage in the left frontal and parietal regions [Figure 1] and [Figure 2]. She was admitted to the intensive care unit and was treated symptomatically with tablet nifedipine 10 mg thrice daily, tablet labetalol 100 mg thrice daily, and intravenous levetiracetam 750 mg. She improved symptomatically and was discharged a week later. On discharge, her BP was 110/70 mmHg, and her systemic examination was normal. She was advised to continue tablet levetiracetam 750 mg. Review MRI brain was done 2 months later which showed multiple fan-shaped areas of blooming involving the right parieto-temporo-occipital regions and left frontal sulcal spaces which were related to the previous subarachnoid hemorrhage; it did not show any features suggestive of PRES that were present earlier [Figure 3].
Figure 1: Magnetic resonance imaging brain, axial view, T2/fluid-attenuated inversion recovery image showing hyperintensity in the bilateral occipital lobe

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Figure 2: Magnetic resonance imaging brain, axial view, T2/fluid-attenuated inversion recovery image showing subarachnoid hemorrhage in the left frontal sulcal space

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Figure 3: Magnetic resonance imaging brain (2 months later), axial view, T2/fluid-attenuated inversion recovery image showing no hyperintensity in the right occipital lobe

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  Discussion Top


PRES is described as a clinical syndrome of insidious onset of headache, confusion or decreased level of consciousness, visual changes, and seizures, which are associated with characteristic neuroimaging findings of posterior cerebral white-matter edema. The various causes for seizures in the postpartum period are eclampsia, cerebral venous thrombosis, meningitis, encephalitis, tuberculoma, and neurocysticercosis.[5]

The pathophysiology of PRES is not clearly understood, but it appears to be related with disordered cerebral autoregulation and endothelial dysfunction, resulting in cytogenic edema of subcortical white matter being a key factor. Cerebral vasoconstriction causing subsequent infarcts in the brain and failure of cerebral autoregulation causing vasogenic edema are also few hypotheses to explain the pathophysiology.[2],[3] Patients usually present with seizures, confusion, raised BP, and visual disturbances. The clinical findings of PRES resemble other neurological conditions such as stroke, cortical venous thrombosis, toxic or metabolic encephalopathy, demyelinating disorders, vasculitis, or encephalitis. Since it most commonly occurs in the postpartum state, it is very important to differentiate eclampsia from PRES since the lines of treatment vary for both.

In eclampsia, patients present with generalized tonic-clonic seizures and hypertension and with any one of the following features: Headache, visual disturbances, proteinuria, thrombocytopenia, raised creatinine levels or liver transaminase levels, or pulmonary edema. Neuroimaging in eclampsia shows features suggestive of petechial cortical hemorrhages or cerebral venous thrombosis.[6],[7]

Rijal et al.[8] reported a similar case associated with eclampsia after 2 weeks of postpartum. Aulakh et al.[9] reported PRES in a 78-year-old African-American man, who presented with increased BP and seizure episodes. In both the cases, MRI was typical of PRES and lesions resolved completely after treatment.

The treatment of PRES is symptomatic; it mainly aims at lowering the BP and preventing further seizures with antiepileptics. It can be totally reversible if diagnosed at the right time, whereas if ignored can lead to fatal complications such as acute hemorrhage and massive posterior fossa edema causing obstructive hydrocephalus or brainstem compression.[10] In a case series, 22 patients were studied and among them 6 died and many who survived had neurological deficit.[11]


  Conclusion Top


PRES is a rare cause of generalized tonic-clonic seizure in postpartum period. It can be associated with conditions such as eclampsia and can mimic cerebral venous thrombosis. It may be completely reversible with early initiation of treatment. Late diagnosis and treatment may cause complications such as acute intracranial hemorrhage, cerebral edema, and even death.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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. 1
    
2.
Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA, et al. Posterior reversible encephalopathy syndrome: Associated clinical and radiologic findings. Mayo Clin Proc 2010;85:427-32.  Back to cited text no. 2
    
3.
Chen TH, Lin WC, Tseng YH, Tseng CM, Chang TT, Lin TJ, et al. Posterior reversible encephalopathy syndrome in children: Case series and systematic review. J Child Neurol 2013;28:1378-86.  Back to cited text no. 3
    
4.
Ay H, Buonanno FS, Schaefer PW, Le DA, Wang B, Gonzalez RG, et al. Posterior leukoencephalopathy without severe hypertension: Utility of diffusion-weighted MRI. Neurology 1998;51:1369-76.  Back to cited text no. 4
    
5.
Jamadarkhana S, Law RC. Seizures in the early post-partum period: A diagnostic dilemma. Indian J Anaesth 2012;56:183-5.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Berhan Y, Berhan A. Should magnesium sulfate be administered to women with mild pre-eclampsia? A systematic review of published reports on eclampsia. J Obstet Gynaecol Res 2015;41:831-42.  Back to cited text no. 6
    
7.
Zeeman GG, Fleckenstein JL, Twickler DM, Cunningham FG. Cerebral infarction in eclampsia. Am J Obstet Gynecol 2004;190:714-20.  Back to cited text no. 7
    
8.
Rijal JP, Giri S, Dawadi S, Dahal KV. Posterior reversible encephalopathy syndrome in a patient with late postpartum eclampsia. BMJ Case Rep 2014;2014. pii: bcr2013203162.  Back to cited text no. 8
    
9.
Aulakh P, Fatakhov E, Koch CF Jr., Kapil S. Posterior reversible encephalopathy syndrome with documented hyponatraemia. BMJ Case Rep 2013;2013. pii: bcr2013009311.  Back to cited text no. 9
    
10.
Ledingham JG, Rajagopalan B. Cerebral complications in the treatment of accelerated hypertension. Q J Med 1979;48:25-41.  Back to cited text no. 10
    
11.
Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: Prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol 2002;23:1038-48.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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