Research Article: Posterior reversible encephalopathy syndrome (PRES) in children – a summary of 20 years of tertiary neurology centre experience
Abstract:
Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome characterised by seizures, hypertension, visual disturbances alongside classical parieto-occipital lesions on neuroimaging. We report the clinical presentation and management of the largest UK paediatric PRES cohort.
Seventy-seven children (x? age?=?8.6?years [SD?=?4.3], 64% male) experienced PRES as inpatients at Great Ormond Street Hospital (London, UK) from January 2005 to May 2024. Electronic patient records were retrospectively analysed.
Most children (97.4%) had significant co-morbidities, including haem-oncological ( n =?25, 32.5%), renal ( n =?17, 22.1%) and immunological conditions ( n =?9, 11.7%). Fifty-four patients (70.1%) took immunomodulating medications. Seizures were the commonest presenting symptom ( n =?60, 77.9%), followed by headaches ( n =?15, 19.4%), visual disturbance ( n =?13, 16.8%) and encephalopathy ( n =?18, 23.3%). Fifty (64.9%) children were hypertensive. Anti-seizure medication (ASM) was commenced in fifty-six children (72.7%), with Levetiracetam ( n =?36; 64.3%) being most used. Thirty-six children (64.3%) required short-term (<6?months) ASMs and twenty (35.7%) remained on long-term treatment (>6?months). Indications for long-term ASMs included persistent seizures ( n =?12) or neurological symptoms ( n =?3) and underlying co-morbidities ( n =?2). Neuroimaging (MRI n =?70 (90.9%); CT n =?7(9.1%)) was performed in all patients. Twenty-four ( n =?24) had the typical parieto-occipital pattern of involvement, whilst forty ( n =?40) children had atypical patterns. Seventy-four ( n =?74, 96%) patients underwent EEG; sixty-seven of these ( n =?67, 90.5%) had abnormal EEG findings. Focal slowing showed a trend towards long-term ASM-use (aOR?=?1.49, 95% CI 0.28–7.88; p =?0.08).
Our cohort showcases the broad clinical spectrum of paediatric PRES. Global standardised clinical pathways are needed to better inform both acute and long-term management and outcomes.
Introduction:
Posterior Reversible Encephalopathy Syndrome (PRES) is a clinico-radiological condition characterised by variable combinations of seizures, altered consciousness, headaches, and visual disturbances ( 1 ). It is also associated with hypertension. Distinctive neuroimaging findings, affecting the parieto-occipital regions of the brain, are also frequently noted ( 2 ). Since its initial description by Hinchey et al. in 1996 ( 3 ), PRES has been increasingly recognised in both adult and paediatric populations, yet its…
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