The Value of Visual Evoked Potential as a Screening Test in Neurofibromatosis

Jabbari, Bahman; Maitland, Charles G.; Morris, Laura M.; J, Morales; Gunderson, Carl H.

doi:10.1001/archneur.1985.04060100054022

Cited by 37 publications

(19 citation statements)

References 5 publications

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“…An abnormal result of visual evoked potential in a child with NF-1 should elicit further investigations with available imaging techniques. The reported high incidence of abnormal visual evoked potential in asymptomatic individuals with optic glioma [3,7,10] and our similar findings support the use of visual evoked potential as a screening test for this neoplasm. Future use of serial visual evoked potentials may help clarify the prevalence and natural history of asymptomatic gliomas which may form a group distinct from those with more potential for destruction of the optic pathways [1,4,8,11,13].…”

Section: Discussionsupporting

confidence: 86%

“…This examination of optic pathway function can be performed either with pattern stimulation, which is the more sensitive mode, or with flash stimulation if the child is too young to co-operate [3,7]. Our results suggest that visual evoked potential is a sensitive, albeit not specific, test for the presence of an optic glioma [3,4,7,10]. An abnormal result of visual evoked potential in a child with NF-1 should elicit further investigations with available imaging techniques.…”

Section: Discussionmentioning

confidence: 81%

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Optic gliomas in children with neurofibromatosis type 1

Lund

Skovby

1991

Eur J Pediatr

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Over a 24-year period, optic gliomas were found in 29 children, 16 of whom had neurofibromatosis type 1 (NF-1). These 16 children comprised 21% of all children referred for management of NF-1 and its complications. The finding of optic glioma led to the diagnosis of NF-1 in 4 children. The mean age at diagnosis of optic glioma in NF-1 children was 6.4 years, and the average estimated duration of visual symptoms prior to diagnosis was 2.1 years. Most optic gliomas in NF-1 children were ascertained because of a visual complaint (69%), and an even greater number of children (88%) had an abnormal ophthalmological examination. The optic chiasm was involved in 75% of the patients. All of the seven children with optic glioma examined by visual evoked potential had an abnormal response ipsilateral to the tumour. The majority of the children received radiation therapy. After a mean follow up period of 5.8 years no deaths had occurred due to optic glioma, but in 35% of the children vision was worse. We conclude that optic glioma is a common, serious complication in NF-1 children. Routine care of such patients should include regular noninvasive investigations aimed at detecting lesions of the optic pathway.

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 81%

Optic gliomas in children with neurofibromatosis type 1

Lund

Skovby

1991

Eur J Pediatr

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“…Visual evoked potentials (VEPs) are an electrophysiologic test believed to provide a functional measure of visual pathway integrity. Although VEP testing may detect OPG with some sensitivity, [42][43][44][45][46] some patients with NF1 have abnormal VEP testing despite no evidence of glioma. 47 Of greater concern is the poor diagnostic sensitivity of VEP for VA loss 48 and the poor correlation of VEP changes over time with VA changes and response to treatment.…”

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confidence: 99%

Functional outcome measures for NF1-associated optic pathway glioma clinical trials

et al. 2013

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Objective: The goal of the Response Evaluation in Neurofibromatosis and Schwannomatosis Visual Outcomes Committee is to define the best functional outcome measures for future neurofibromatosis type 1 (NF1)-associated optic pathway glioma (OPG) clinical trials. Methods:The committee considered the components of vision, other ophthalmologic parameters affected by OPG, potential biomarkers of visual function, and quality of life measures to arrive at consensus-based, evidence-driven recommendations for objective and measurable functional endpoints for OPG trials.Results: Visual acuity (VA) assessments using consistent quantitative testing methods are recommended as the main functional outcome measure for NF1-OPG clinical trials. Teller acuity cards are recommended for use as the primary VA endpoint, and HOTV as a secondary endpoint once subjects are old enough to complete it. The optic disc should be assessed for pallor, as this appears to be a contributory variable that may affect the interpretation of VA change over time. Given the importance of capturing patient-reported outcomes in clinical trials, evaluating visual quality of life using the Children's Visual Function Questionnaire as a secondary endpoint is also proposed. Conclusions:The use of these key functional endpoints will be essential for evaluating the efficacy of future OPG clinical trials. Neurology ® 2013;81 (Suppl 1):S15-S24 GLOSSARY CVFQ 5 Children's Visual Function Questionnaire; logMAR 5 logarithm of the minimum angle of resolution; MS 5 multiple sclerosis; NF1 5 neurofibromatosis type 1; OCT 5 optical coherence tomography; OPG 5 optic pathway glioma; PFS 5 progression-free survival; QOL 5 quality of life; REiNS 5 Response Evaluation in Neurofibromatosis and Schwannomatosis; RNFL 5 retinal nerve fiber layer; TAC 5 Teller acuity cards; VA 5 visual acuity; VEP 5 visual evoked potential; VF 5 visual field.Optic pathway gliomas (OPG) arise in 15%-20% of children with neurofibromatosis type 1 (NF1), occur preferentially in young children compared with adolescents or adults, and cause vision loss in as many as half of those affected.1 In this regard, the main objective in clinical management of these tumors is preservation of visual function. Although prognostic factors have been identified, there are currently no reliable indicators of future visual loss. This absence of prognostic signs has led clinicians to avoid initiating treatment until visual function has declined. When treatment is indicated, NF1-OPG are typically managed with a combination of carboplatin and vincristine-an approach that has not changed in 15 years. 2To date, OPG clinical trials have focused on imaging outcomes, with tumor response and/or progression-free survival used as measures of treatment success.

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“…Several authors have reported flash and/or pattern visual evoked potentials (VEPs) in the objective and non-invasive functional testing in patients with OGs [1][2][3][4][5][6][7], as earlier diagnosis allows closer monitoring of the patient, and earlier detection of visual pathway dysfunction. Pattern VEPs are more sensitive than flash VEPs [5].…”

Section: Introductionmentioning

confidence: 99%