Posterior reversible encephalopathy syndrome (PRES) is a rare neurological entity, typically manifested by reversible oedema in the parieto-occipital lobes. It is usually associated with primary hypertension, autoimmune diseases and immunosuppressants. Renal disease is an uncommon cause of PRES. We report a case of an 11-year-old boy with STimulator of INterferon Genes-associated vasculopathy with onset in infancy complicated by focal segmental glomerulosclerosis leading to hypertension and PRES. The patient presented with headache, acute bilateral visual loss and hypertension. Brain MRI showed atypical features revealed by parieto-occipital haemorrhage. The child improved few days after antihypertensive therapy. Follow-up MRI showed complete resolution of haemorrhage. It is important to keep high index of suspicion for the uncommon association of PRES with underlying kidney disease with or without immunosuppressive agents. This combination is the first to our knowledge to be described in paediatric population. Atypical MRI features such as haemorrhage should be kept in mind. Symptoms are reversible within days to weeks with early diagnosis and treatment.
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