G118 REVIEW OF POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME

D. Peake, S. Carter, S. Philip, L. MacPherson, E. Wassmer.Birmingham Children’s Hospital, Birmingham, UK

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a recently recognised neurological syndrome¹ that mainly affects the cerebral white matter and characteristically shows diffuse hyperintensity predominantly involving the parieto-occipital white matter on T2 weighted MRI. It has a rapidly evolving clinical course.

Aim: To review retrospectively presentation, aetiology, intervention, and outcome of all cases of PRES admitted to Birmingham Children’s Hospital from 1997–2005.

Method: Children with a diagnosis of PRES were ascertained retrospectively from the hospital radiology and paediatric neurology databases. Clinical notes and neuroimaging were reviewed.

Results: Seven children (3 male:4 female) aged 0.5 to 13 years (mean 6.5 years) had a clinical and radiological diagnosis consistent with PRES. Two patients had acute lymhoblastic leukaemia, two were post liver transplantation, and three patients had underlying renal disease. The aetiology in four cases was associated with hypertensive encephalopathy, two with immunotherapy (tacrolimus and methotrexate), and one with a combination of hypertension and tacrolimus. Mean time to diagnosis was within 24 hours (range 1–3 days). Seizures and visual disturbances were present in 86% (6/7) Agitation and focal deficits were documented in 57% (4/7) and headache in 43% (3/7). All patients had an altered sensorium at the time of presentation. Control of blood pressure or withdrawal of immunosuppressive/cytotoxic agents caused reversal of the syndrome in all patients. Mean time to recovery was seven days. Residual neurological deficits were documented in two patients. One child died two months after the event. The cause of death was not determined.

Conclusion: PRES is a rare neurological disorder that should be considered in patients with rapidly evolving symptoms of visual disturbance, encephalopathy, and seizures, especially in the “at risk” group—that is, a history of renal disease and hypertension and those on …