MNJ (Malang Neurology Journal) Vol. 10, No. 1, January 2024
Page 76 of 3 http://mnj.ub.ac.id/
DOI: 10.21776/ub.mnj.2024.010.01.16 eISSN: 2442-5001 pISSN: 2407-6724
Accredited by DIKTI Decree No: 21/E/KPT/2018
A RARE CAUSE OF ENCEPHALOPATHY IN CHILDREN:
PRES SYNDROME
Demet Tosun, Nihal Akçay1, Mustafa Oğur1, Esra Şevketoğlu1 Correspondence: [email protected]
1Department of Pediatric Intensive Care Unit, Bakırköy Dr. Sadi Konuk Research and Training Hospital, University of Health Sciences, Istanbul, Turkey.
Article History:
Received: December 4, 2022 Accepted: October 13, 2023 Published: January 1, 2024
ABSTRACT
Background: Major clinical symptoms of posterior reversible encephalopathy syndrome include headache, mental status changes, visual disturbances, seizures, especially in the posterior cerebral region, and radiographic findings of vasogenic edema, especially in the posterior cerebellar region.
Case Report: We present our 6-year-old patient with chronic renal failure. admitted to our pediatric emergency department with an acute hypertensive crisis. Generalized tonic-clonic seizures and mental status changes were accompanied in the follow-up of the patient whose GCS was 11; With symptomatic treatment, regression in both clinical complaints and imaging findings of our patient is consistent with Posterior reversible encephalopathy syndrome (PRES).
Conclusion: It is imperative to consider PRES syndrome as a differential diagnosis when pediatric patients exhibit symptoms of acute encephalopathy. It is crucial to emphasize the critical nature of early diagnosis and treatment, as well as the potential for complete recovery without sequelae.
Keywords: Encephalopathy, children, PRES syndrome Cite this as:
Tosun D, Akçay N, Oğur M, Şevketoğlu E. A rare cause of encephalopathy in children: Pres syndrome. Malang Neurology Journal; 2024.10:76-78. DOI:
http://dx.doi.org/10.21776/ub.mnj .2024.010.01.16
Introduction
Clinical symptoms of posterior reversible encephalopathy syndrome include typical radiographic findings of vasogenic edema, especially in the posterior cerebral region, which include headache, mental status changes, visual disturbances, and seizures.
Although the pathophysiology is still unclear, it can be seen in the background of renal pathologies, autoimmune diseases, or malignancies.1,2 In addition, triggering factors such as hypertension, cytotoxic agents or corticosteroid use may accompany it.3,4
In studies conducted in the pediatric patient group, the mean age was found to be 10-11 years, and after rapid diagnosis with magnetic resonance imaging (MRI), it progresses with complete clinical recovery radiologically. Seizures, hypertension, and atypical neuroimaging findings are the most common findings in childhood posterior reversible encephalopathy syndrome.5
In this case report, the reversible symptoms and characteristic imaging findings of our 6-year-old patient who presented with the diagnosis of end-stage renal disease due to nephrotic syndrome, vomiting, fever, hypertension, and generalized tonic-clonic seizures led us to the diagnosis of PRES.
Case Report
6-year-old male patient, prenatal follow-up, uneventful; He was born at natal term with 3000 g and was diagnosed with
nephrotic syndrome at the age of 2, and peritoneal dialysis is applied with the diagnosis of end-stage renal disease.
When she applied to the pediatric emergency department of our hospital with complaints of fever, vomiting, headache, and clouding of consciousness.
that lasted for about 2 days, the child was followed up for observation, and she was admitted to our pediatric intensive care unit with a preliminary diagnosis of hypertensive crisis since she had recurrent generalized tonic-clonic seizures and blood pressure values were above the 97th percentile for age.
On physical examination, the patient is non-cooperative, non-orientated, GCS: 11, pretibial edema and periorbital edema are present especially in the lower extremities, respiratory sounds are bilaterally equal/natural, fever 37 C, arterial blood pressure is 180/110, pulse: 110, respiratory minute rate: 20. In the abdominal examination, bowel sounds were increased and there was no defensive rebound.
His white blood cell (per μL): was 13700, Hemoglobin (g/dL): was 7.7, Platelet (per μL): was 170.000, urinalysis had protein ++, PT, PTT, INR, liver enzymes were normal, and CRP-Procalcitonin was within the limits. Cranial MRI findings were consistent with PRES. Signal changes were observed in the T1W hypointense and T2W -FLAIR sequences in the right and left temporal, left posterolateral mesencephalon, and left posterolateral side of the mesencephalon, and both insular cortex levels more prominently on the left, which also affect the frontal lobe level towards the high convexity level. There are gyral thickenings at this level.
CASE REPORT OPEN ACCESS
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MNJ (Malang Neurology Journal) Vol. 10, No. 1, January 2024
Figure 1. A slight glare was observed on diffusion-weighted images at the levels described, and there were appearances in ADC of hypointense restriction-like areas (A). Diffusion restriction disappeared in control imaging (B).
A slight glare was observed on diffusion-weighted images at the levels described, and there were appearances in the ADC of hypointense restriction-like areas. The patient's imaging findings are shown in Figure 1.
Sudden headache, hypertension, ischemic stroke, cerebral hemorrhage and cerebral venous thrombosis, PRES, and hypertensive emergency were considered. Fever and clouding of consciousness were considered prediagnoses of infective encephalitis, autoimmune or paraneoplastic encephalitis, and ADEM. The characteristic MR findings and the regression of symptoms led us to the diagnosis of PRES.
He was admitted to the intensive care unit. He was followed up on a cardiopulmonary monitor. Esmolol infusion was started at 200 mcg/kg/h, and
nicardipine infusion was started at 0.5 mcg/kg/h. The drugs he used: Levetiracetam 40 mg/kg/day,
AntiPhosphate CC 2x250 mg and Folic acid 1X1 oral tablet were continued. Cefepime was added due to possible peritonitis. Peritonitis was ruled out with the result of peritoneal cell count on the 2nd day of hospitalization.
Antibiotherapy was stopped. Oral antihypertensives were started in the patient with oral tolerance, and IV antihypertensives were discontinued on the 4th day of hospitalization, according to blood pressure follow-ups.
Discussion
PRES is characterized by focal neurological symptoms, migraines, seizures, encephalopathy, and visual disturbances.6. One of the defining characteristics of the disease is the reversibility of these symptoms, as their name implies. Nevertheless, severe PRES manifestations,
including hypertensive crisis and status epilepticus, may necessitate intensive care unit follow-up for certain patients.7,8
Early diagnosis, symptomatic treatment, and cause-directed treatment are all components of Pres management.7,8. As the name suggests, appropriate treatment is expected to lead to full recovery. However, persistent complications and deaths have been reported.
Conclusion
On the 7th day of hospitalization, the neurological symptoms regressed, the headache did not disappear, the consciousness did not disappear, and the patient had a normotensive course.
A control cranial MRI was taken: the previously existing lesions were observed to have regressed significantly, and the diffusion restriction regressed.
In conclusion, It is imperative to consider PRES syndrome as a differential diagnosis when pediatric patients exhibit symptoms of acutely developing encephalopathy. It is crucial to emphasize the critical nature of early diagnosis and treatment, as well as the potential for complete recovery without sequelae. The gold standard for diagnosis is cranial MRI, and treatment is agent-specific. It is imperative to bear in mind that irreversible brain injury and neurological complications may result from postponing treatment.
Acknowledgement
None.
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Conflict of Interest
There is no conflict of interest.
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