Giant pattern VEPs in children

Dorothy A Thompson; Oliver R Marmoy; Katrina L Prise; Victoria M Reynolds; Sian E Handley; Dominique M Versace; Sina Kafiabadi; Kshitij Mankad; Vasiliki Panteli; Kemmy Schwiebert; Greg James; Richard Bowman

doi:10.1016/j.ejpn.2021.07.008

Giant pattern VEPs in children

Eur J Paediatr Neurol. 2021 Sep:34:33-42. doi: 10.1016/j.ejpn.2021.07.008. Epub 2021 Jul 30.

Affiliations

¹ Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, WC1N 3JH, UK; Ulverscroft Vision Research Group, UCL Great Ormond Street Institute for Child Health, 30 Guilford Street, London, WC1N 1EH, UK. Electronic address: Dorothy.Thompson@gosh.nhs.uk.
² Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, WC1N 3JH, UK; Ulverscroft Vision Research Group, UCL Great Ormond Street Institute for Child Health, 30 Guilford Street, London, WC1N 1EH, UK.
³ Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, WC1N 3JH, UK.
⁴ Neuroradiology Department, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, WC1N 3JH, UK.
⁵ Department of Neurosurgery, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, WC1N 3JH, UK; Craniofacial Research Group, UCL Great Ormond Street Institute of Child Health, 30 Guilford Street, London, WC1N 1EH, UK.

PMID: 34388649
DOI: 10.1016/j.ejpn.2021.07.008

Abstract

Our aim is to elaborate the clinical significance of giant amplitude pattern reversal visual evoked potentials (VEPs) in children. 'Giant' amplitude VEPs exceed the upper 97.5^th centile, 90% CI for age. We scrutinised 2750 pattern VEPs recorded to international standards between Jan 2015 and 2017 from children aged 16 years and under, attending a specialist children's hospital. Twenty seven children, median age 6yrs, (range 1-16 yrs), were identified with giant VEPs (P100 amplitude range 65-163 μV). Most, 22/27 (81%), had conditions associated with a risk of raised ICP. Sixteen of these twenty two children had craniosynostosis; six multi-sutural and eight single suture disease. Others had Idiopathic Intracranial Hypertension, arachnoid cyst, NF1 with shunted hydrocephalus, chronic infantile neurological cutaneous and articular (CINCA) syndrome, nephrotic cystinosis and obstructive sleep apnoea. Five children presented with a range of conditions, some associated with seizures some symptomatic, but as yet undiagnosed. Frequent structural associations were optical coherence tomography measures of optic disc maximum anterior axial horizontal retinal thickness projection >160 μm and neuro-radiological findings of CSF effacement and copper beaten appearance. Ultrasonography measures of optic nerve sheath diameters varied, but in one child took 2 years to resolve after treatment for raised ICP. Optic disc gradings by fundoscopy were mostly normal, as were visual acuities. Raised ICP was confirmed by gold standard ICP bolt measurements in five of seven children tested. These data suggest that rICP should be considered if a child has sustained giant amplitude VEPs at normal latency.

Keywords: Craniosynostosis; Giant amplitude; Intracranial hypertension; Raised intracranial pressure; VEP; Visual evoked potential; rICP.

MeSH terms

Adolescent
Child
Child, Preschool
Evoked Potentials, Visual
Humans
Hydrocephalus*
Infant
Optic Disk*
Pseudotumor Cerebri*
Visual Acuity

Grants and funding

MR/T005297/1/MRC_/Medical Research Council/United Kingdom