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Posterior reversible encephalopathy syndrome associated with focal segmental glomerulosclerosis in a child
Nadine Nassar1,2, Charbel Chater3,4, Amal Chelala1,2
1Department of Radiology, Notre Dame des Secours University Hospital Center, Jbeil, Mont-Liban, Lebanon.
Insights
Posterior reversible encephalopathy syndrome (PRES), a rare neurological condition, can be caused by kidney disease. This case highlights PRES with haemorrhage in a child with STING-associated vasculopathy and focal segmental glomerulosclerosis.
Area of Science:
- Neurology
- Nephrology
- Pediatrics
Background:
- Posterior reversible encephalopathy syndrome (PRES) is a rare neurological disorder characterized by reversible parieto-occipital lobe edema.
- PRES is typically linked to hypertension, autoimmune conditions, and immunosuppressants, with renal disease being an infrequent cause.
- STimulator of INterferon Genes (STING)-associated vasculopathy with onset in infancy (SAVOIN) is a rare autoinflammatory disease.
Observation:
- A case report of an 11-year-old boy with SAVOIN complicated by focal segmental glomerulosclerosis (FSGS) leading to hypertension and PRES.
- The patient presented with headache, acute bilateral visual loss, and severe hypertension.
- Brain MRI revealed atypical features, including parieto-occipital haemorrhage, in addition to edema.
Findings:
- The child's symptoms and hypertension significantly improved following antihypertensive therapy.
- Follow-up MRI demonstrated complete resolution of the cerebral haemorrhage.
- This case represents the first reported instance of PRES associated with SAVOIN and FSGS in a pediatric patient.
Implications:
- Highlights the importance of considering uncommon associations between PRES and underlying kidney disease, even in the absence of immunosuppressive agents.
- Emphasizes the need for a high index of suspicion for PRES in pediatric patients with vasculopathy and renal compromise.
- Underscores that atypical MRI findings, such as haemorrhage, can occur in PRES and that early diagnosis and treatment lead to reversible outcomes.
Abstract:
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.
