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PurposeTo highlight the importance of simultaneous flash electroretinogram (ERG) and visual evoked potential (VEP) recording to differentiate a true flash VEP response from an artefact caused by the intrusion of the ERG on a mid-frontal reference electrode in cases of severe cerebral visual impairment (CVI).MethodsWe report an observational case series of four children with severe CVI who underwent simultaneous flash ERG and VEP recordings. Flash VEPs from Oz–Fz and lower lid skin ERGs referred to Fz were recorded simultaneously to Grass intensity setting 4 flash stimulation.ResultsIn all cases, atypical, but reproducible VEPs were evident. Comparison of the timing and waveform of the VEPs and ERGs showed the occipital responses were inverted ERGs and no true flash VEP was evident.ConclusionsWhile ISCEV and neurophysiology standards do not require the simultaneous recording of the flash ERG with the VEP, these cases highlight the usefulness of this non-invasive technique particularly in suspected paediatric cerebral visual impairment to differentiate a true VEP from an artefact caused by ERG contamination.

Background/AimsOptic pathway gliomas (OPGs) may cause progressive visual loss despite chemotherapy. Newer, less toxic treatments might be given earlier, depending on visual prognosis. We aimed to investigate the prognostic value of visual evoked potentials (VEP) and optical coherence tomography (OCT). MethodsA retrospective study of OPG patients (treated 2003-2017) was conducted. Primary outcome was PEDIG category visual acuity in better and worse eyes (good <= 0.2, moderate 0.3-0.6 and poor >=0.7 logMAR). Binary logistic regression analysis was used to identify predictors of these outcomes. Results60 patients (32 Neurofibromatosis type 1 [NF1] and 28 sporadic) had median presentation age 49 months (range 17-183) (NF1) and 27 months (range 4-92) (sporadic). Median follow up was 82 months (range 12-189 months). At follow up 24/32 (75%) of NF1 children and 14/28 (50%) of sporadic children had good better eye visual acuity and 11/32 (34%) of NF1 children and 15/28 (54%) of sporadics had poor worse eye acuity. Mean peripapillary retinal nerve fibre layer (RNFL) thickness predicted good better eye final acuity (OR 0.799, 95%CI 0.646-0.987, p=0.038). Presenting with visual symptoms (OR 0.22 95% CI 0.001-0.508, p=0.017) and poorer VEP scores (OR 2.35 95% CI 1.1-5.03, p=0.027) predicted poor worse eye final acuity. 16 children had homonymous hemianopias at follow up, predicted by poor presenting binocular VEP score (OR 1.449 95%CI 1.052-1.995, p=0.02). ConclusionsWe found that both RNFL thickness on OCT and VEP were useful in predicting future visual acuity and visual field and potentially in planning treatment. We had a high prevalence of homonymous hemianopia.

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder predominantly affecting the peripheral nervous system (PNS), characterised by muscle weakness and sensory disruption which may involve all four limbs[1]. CIDP affecting children is uncommon, with a large scale review in 2013 noting 143 documented cases described in the literature [2]. Central nervous system involvement has previously been described; however, optic neuropathy is an extremely rare manifestation of CIDP, with only a small number of cases previously reported (both unilateral and bilateral) in adult patients[3-5]. This report details a child who developed optic neuropathy in association with CIDP.