Posterior reversible encephalopathy syndrome (PRES) in children - a summary of 20 years of tertiary neurology centre experience.

OBJECTIVE

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.

METHODS

Seventy-seven children (x̄ 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.

RESULTS

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).

CONCLUSION

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.