POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME (PRES)- CASE REPORT & REVIEW OF LITERATURE

Abstract

A seven-year-old male child presented with generalised progressive swelling all over body with decreased urine output and nephrotic range proteinuria. He had history of headache & visual impairment since 6 months. No history of fever, arthralgia, rashes or any other symptoms present. His blood pressure was 160/90 mmHg. Investigations: Hb-6.37 gm/dl, TLC-20,110/cmm, TPC-4.1 lakh/cmm, Sr. Urea-63 mg/dl, Sr. Creatinine-1.1 mg/dl, Sr. Total Protein-3.7 gm/dl, Serum albumin-1.4 gm/dl, Total cholesterol-351 mg/dl, ANA-1+Positive, ASO-Negative, Sickling test-Negative, Sr. C3-78 mg/dl, HIV/HBsAg-Negative, Urine RE/ME-Albumin 4+, pus cells-0-2/HPF, RBCs-4-6/HPF, hyaline cast-0-2/LPF, Spot urine protein-814.8 mg/dl, spot urine creatinine-32.15 mg/dl, Urine protein creatinine ratio-25.343 mg/gm, Stool RE/ME-Strongyloides stercoralis, USG Abdomen &pelvis showed cholelithiasis, ascites. MRI brain revealed bilateral occipital cortex cortical atrophy. Clinical diagnosis was Nephrotic syndrome associated PRES. Renal percutaneous biopsy was done. Histopathology showed presence of fetal glomeruli & mild mesangial matrix accentuation in one glomerulus. (Fig. 1, 2) Features of acute tubular injury with atrophic tubules seen with moderate chronic inflammatory cell infiltration in interstitium. (Fig. 3) Blood vessels were unremarkable. Immunofluorescence study showed minimal positivity for IgM& C3 antisera. So, the diagnosis of tubulointerstitial nephritis with mild mesangial sclerosis in association with PRES was given. DIFFERENTIAL DIAGNOSIS The non-specific clinical manifestations and multiplicity of radiological patterns increases the diagnostic challenges as various other conditions mimic PRES. 12 1. Acute Cerebral Ischaemia: Unilateral lesions with a decreased diffusion coefficient. 2. Cerebral Venous Thrombosis: Asymmetric, diffuse lesions. 3. Transient Cerebral Hyperaemia: Develops after an episode of seizure or end arterectomy.

Posterior reversible encephalopathy syndrome (PRES) in a 6-year-old child with nephrotic syndrome

“…Acute leukemia, glomerulonephritis, hemolytic uremic syndrome, Henoch-Schonlein Purple, use of cytotoxic drugs are closely associated with PRES in children. The clinical and radiological characteristics of PRES in children are similar to those of adults, according to few small case studies and case reports available [17] . From published studies it seems that the incidence is between 0.04% and 5.2% [10] , [11] , [12] , 17] .…”

“…Seizures are the most common presentation symptom (60%-75%) followed by encephalopathy (50%-80%), visual disturbances (33%), headache (50%) and focal neurological deficits (10%-15%) [9 , 17 , 21] .…”

“…PRES is known to have a favorable outcome, as most patients fully recover within a week, although some may take longer [13 , 17 , 23 , 26] . Recurrent PRES was observed in 4% of patients in retrospective studies [12] , [25] , [27] .…”