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G370(P) Posterior Reversible Encephalopathy Syndrome in children with Nephrotic Syndrome: A report of 2 cases
  1. GS Dhooria,
  2. HS Bains,
  3. PA Pooni,
  4. D Bhat
  1. Department of Pediatrics, Dayanand Medical College & Hospital, Ludhiana, Punjab, India

Abstract

Introduction Posterior reversible leukoencephalopathy syndrome (PRES) clinically can present with seizures, headache, mental and visual changes. We report two cases of nephrotic syndrome who developed clinical and neuro-imaging signs of PRES on high dose steroids. Both cases had some additional renal disease. One had single kidney and renal dysfunction where as other one had pyelonephritis.

Case 1: An 11-years old boy with Steroid dependent nephrotic syndrome on high dose steroids since 6 months presented in Pediatric Emergency with seizures and altered sensorium since one day. He had over feet and face, easy bruisablity, short stature. Blood pressure was 170/110 mm Hg with raised creatinine and hypoalbuminemia. Metabolic profile was normal. Ophthalmological examination revealed posterior subcapsular cataract and Grade 4 hypertensive retinopathy. Magnetic resonant imaging Brain was suggestive of PRES. Renal Ultrasound revealed left single kidney. DMSA scan showed non-functional/ non visualised right kidney with reduced cortical functioning on left kidney with no scars (Figure 1).

Case 2: A 9 years old boy diagnosed as a case of first episode of nephrotic syndrome 3 weeks back and was on full dose steroids. Child presented in Paediatric Emergency with seizures and altered sensorium since one day. There was no edema. Blood pressure was 140/100 mm Hg with normal renal functions. Ophthalmological examination and metabolic profile was normal. Urine routine showed 1+ albumin and 20–25 pus cells/hpf. Leucocytosis was present and CRP was positive. Renal Ultrasound revealed enlarged kidneys with renal parencymal disease. Magnetic resonant imaging brain was suggestive of PRES (Figure 2). Urine culture was positive for E coli. He also developed features of Steroid induced psychosis for which anti-psychotic medications were started.

Conclusions We suggest that PRES should be suspected in children with Nephrotic syndrome with sudden onset of neurological signs and symptoms on high dose steroids and underlying cause for hypertension should be evaluated.

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