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Year : 2021  |  Volume : 5  |  Issue : 2  |  Page : 90-92

Posterior reversible encephalopathy syndrome: An acute neurological complication of blood transfusion

Department of Neurology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India

Date of Submission29-Sep-2020
Date of Acceptance05-May-2021
Date of Web Publication23-Jul-2021

Correspondence Address:
Dr. Madhavi Karri
Department of Neurology, PSG Institute of Medical Sciences and Research, PSG Hospitals, Peelamedu, Coimbatore - 641 004, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ljms.ljms_89_20

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Posterior reversible encephalopathy syndrome (PRES) is an acute neurological condition with characteristic clinical and radiological features. PRES occurring as a complication post blood transfusion is reported to be rare, irrespective of the presence of an underlying hematological disorder. Here, we describe a case of a 37-year old female known case of chronic anemia secondary to menorrhagia, who received three units of packed red blood cells transfusion and there was raise in hemoglobin from 6 gm/dl to 11 gm/dl. Four hours after the last transfusion, she developed occipital headache with neck pain and presented to us with two episodes of generalized tonic-clonic seizures after four days of symptoms onset. With this clinical picture in correlation with magnetic rsonance imaging findings, features were consistent with PRES. PRES is contemplated as one of the acute transfusion reactions but a rare entity, which is often preventable.

Keywords: Anaemia, blood transfusion, posterior reversible encephalopathy syndrome

How to cite this article:
Karri M, Ramasamy B, Perumal S, Kannan KT. Posterior reversible encephalopathy syndrome: An acute neurological complication of blood transfusion. Libyan J Med Sci 2021;5:90-2

How to cite this URL:
Karri M, Ramasamy B, Perumal S, Kannan KT. Posterior reversible encephalopathy syndrome: An acute neurological complication of blood transfusion. Libyan J Med Sci [serial online] 2021 [cited 2023 Mar 30];5:90-2. Available from: https://www.ljmsonline.com/text.asp?2021/5/2/90/322209

  Introduction Top

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome characterized by headache, seizures, visual disturbances, and confusion. Magnetic resonance imaging (MRI) findings typically involve posterior regions with subcortical vasogenic edema. It was first described in 1996 by Hinchley et al.[1] Several causes are associated with PRES, among which accelerated hypertension been most common for causation of PRES. PRES secondary to blood transfusion is rare. There were very few cases reported posttransfusion PRES and most of them are associated with underlying preexisting hematological disorders.[2],[3] Here, we report a case of PRES occurring secondary to blood transfusion in a female with microcytic hypochromic anemia secondary to menorrhagia and no underlying primary hematological disorder.

  Case Report Top

A 37-year-old female presented with sudden-onset occipital headache with neck pain for 5 days. Then, she developed two episodes of generalized tonic seizures with tongue bite on July 29, 2019 for which she came to our hospital. Her history revealed she had microcytic hypochromic anemia (secondary to massive menstrual bleed) for 3 years, not on regular supplements. She received three units of packed red cells transfusion on July 24, 2019, after which there was a rise in hemoglobin from 6 gm/dl to 11 gm/dl. Four hours following the transfusion, she developed the above symptoms. Clinically, she was conscious, afebrile with a Glasgow coma scale of 15/15 and normal blood pressure of 140/90 mm of Hg. She had terminal neck stiffness with the rest of the neurological examination showed no focal neurological deficits. Her magnetic resonance imaging (MRI) of the brain with contrast showed T2 and Fluid attenuated inversion recovery (FLAIR) showed hyperintensities in left occipital [Figure 1]a, bilateral parietal [Figure 1]b and bilateral frontal [Figure 1]c gray matter without diffusion restriction which was consistent with posterior reversible encephalopathy syndrome (PRES). There was no abnormal contrast enhancement and magnetic resonance venogram imaging showed no evidence of abnormality.
Figure 1: Magnetic resonance imaging of the brain with contrast with T2 and fluid attenuated inversion recovery showing hyperintensities in left occipital (a), bilateral parietal (b) and bilateral frontal (c) gray matter which was consistent with posterior reversible encephalopathy syndrome

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Blood investigations showed normal sugars, renal and liver function tests. Cerebrospinal fluid analysis was normal. Electroencephalogram showed focal spike discharges over the right posterior head region. She received a loading dose of levetiracetam and later continued with the maintenance dose. Besides, she also received analgesics as needed for headache. She improved symptomatically and got discharged in three days. Follow up MRI brain scan done a month later, showed clearance of radiological features.

  Discussion Top

PRES is a reversible and treatable neurological condition, associated with a good prognosis. About 70%–80% are known to occur secondary to uncontrolled hypertension. Other nonhypertensive conditions include chronic renal failure, eclampsia/preeclampsia, autoimmune, and posttransplantation. The most common pathogenesis is disruption of autoregulation causing vasodilatation and hyperperfusion, causing cerebral damage, or uncontrolled vasoconstriction causing hypoxia and vasogenic edema. The other mechanism is vascular endothelial dysfunction due to systemic inflammation causing cytotoxic and vasogenic edema.[4]

MRI in PRES shows T2-FLAIR hyperintensities typically in bilateral parietooccipital regions, holo-hemispheric, and superior frontal sulcus pattern with no diffusion restriction.[5],[6] It is usually bilateral and symmetrical in involvement. Brainstem and basal ganglia are affected in about 30% of cases. Atypical areas such as the brainstem, basal ganglia, and thalamus are also involved with increasing frequency. In 10%–20% diffusion restriction is noticed secondary to severe irreversible tissue damage by cytotoxic edema. Early recognition and treatment promptly cause resolvement of both clinical and radiologic features within days to weeks.

In chronic anemia, rapid correction with large volumes of blood can induce PRES. It occurs due to disruption of autoregulation, and hyperperfusion, and hyper-oxygenation, causing cerebral damage.[7] Due to increase viscosity of blood and impaired long-standing hypoxia-induced vasodilatation, causing an increase in vascular resistance. It is seen more commonly in females though the cause was not known. Similar cases were reported in the USA,[8] Korea[9] and a case series in Japan.[10] PRES is considered an acute neurological complication after a massive blood transfusion, which can be prevented and preserve the quality of life.

  Conclusion Top

Postblood transfusion-related PRES is rare and a less reported entity. Here, in this case, there was impairment of autoregulation following packed red cell transfusion causing a rapid rise in blood viscosity, in chronic anemia individuals with radiological features consistent with PRES. Hence, monitored blood transfusion is to be planned accordingly to prevent its occurrence and maintain a better quality of life.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Hinchley J, Chaves C. A reversible posterior leukoencephalopathy syndrome. N Eng J Med 1996;334:494-500.  Back to cited text no. 1
Verma A, Hemlata, Elhence P, Phadke SR, Neyaz Z. Posterior reversible encephalopathy syndrome following blood transfusion in a patient with factor X deficiency: Is it an unusual systemic manifestation of an adverse transfusion reaction? Transfus Apher Sci 2018;57:50-3.  Back to cited text no. 2
Kolovou V, Zampakis P, Ginopoulou A, Varvarigou A, Kaleyias J. Reversible posterior leukoencephalopathy syndrome after blood transfusion in a pediatric patient with sickle cell disease. Pediatr Neurol 2013;49:213-7.  Back to cited text no. 3
Bartynski WS. Posterior reversible encephalopathy syndrome, part 2: Controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol 2008;29:1043-9.  Back to cited text no. 4
Hugonnet E, Da Ines D, Boby H, Claise B, Petitcolin V, Lannareix V, et al. Posterior reversible encephalopathy syndrome (PRES): Features on CT and MR imaging. Diagn Interv Imaging 2013;94:45-52.  Back to cited text no. 5
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. 6
Ito Y, Niwa H, Iida T, Nagamatsu M, Yasuda T, Yanagi T, et al. Post-transfusion reversible posterior leukoencephalopathy syndrome with cerebral vasoconstriction. Neurology 1997;49:1179-5.  Back to cited text no. 7
Singh K, Gupta R, Kamal H, Silvestri NJ, Wolfe GI. Posterior reversible encephalopathy syndrome secondary to blood transfusion. J Clin Neurosci 2015;22:592-4.  Back to cited text no. 8
Heo K, Park S, Lee JY, Lee BI, Lee SK. Post-transfusion posterior leukoencephalopathy with cytotoxic and vasogenic edema precipitated by vasospasm. Cerebrovasc Dis 2003;15:230-3.  Back to cited text no. 9
Nakamura Y, Sugino M, Tsukahara A, Nakazawa H, Yamamoto N, Arawaka S. Posterior reversible encephalopathy syndrome with extensive cytotoxic edema after blood transfusion: A case report and literature review. BMC Neurol 2018;18:190.  Back to cited text no. 10


  [Figure 1]

This article has been cited by
1 Posterior reversible encephalopathy syndrome secondary to blood transfusion
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Libyan Journal of Medical Sciences. 2021; 5(4): 177
[Pubmed] | [DOI]


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