Electrophysiological assessment for early detection of retinal dysfunction in ß-thalassemia major patients.

PURPOSE: The purpose of this study was to assess the role of various diagnostic tests in early detection of retinal changes in ß-thalassemia major patients. METHODS: Thirty-eight visually asymptomatic ß-thalassemia major patients receiving regular blood transfusions and iron-chelation therapy with deferoxamine (group A, n = 13), deferasirox (group B, n = 11) or deferoxamine with deferiprone (group C, n = 14) and fourteen age- and sex- matched healthy individuals were included in the study. All participants underwent ophthalmoscopy, full-field electroretinography (ERG), visual evoked potentials (VEP), multifocal electroretinography (mfERG), fundus autofluorescence (FAF) imaging and optical coherence tomography (OCT) scans. RESULTS: Retinal pigment epithelium changes were present in two cases. Scotopic ERG demonstrated decreased a-wave amplitude in groups A, B and C (p = 0.03, p = 0.002 and p = 0.002, respectively) and decreased b-wave amplitude in groups B and C (p = 0.002 and p = 0.01, respectively) compared to controls. Photopic ERG showed delayed b-wave latency in groups A and C (p = 0.03 and p = 0.03, respectively) ERG maximal combined response and VEP response did not differ between groups. MfERG showed reduced retinal response density in ring 1 in groups A, B, C (p < 0.001, p < 0.001, p = 0.001, respectively) and ring 2 in group B (p = 0.02) and delayed latency in ring 5 in groups A and B (p = 0.04 and p = 0.04, respectively). Abnormal FAF images appeared in three cases and OCT abnormalities in one case, whereas no changes were observed in controls (p = 0.55 and p = 1.00, respectively). CONCLUSIONS: Full-field ERG and mfERG are more sensitive tools for detecting early retinal changes in ß-thalassemia patients compared with ophthalmoscopy, VEP, FAF imaging and OCT scans.

The Role of Multifocal Electroretinography in the Assessment of Drug-Induced Retinopathy: A Review of the Literature.

Multifocal electroretinography (mfERG) is an objective, noninvasive examination for the assessment of visual function. It enables the stimulation of multiple retinal areas simultaneously and recording of each response independently, providing a topographic measure of retinal electrophysiological activity in the central 40-50° of the retina. A clinical application of mfERG represents the assessment of retinal toxicity associated with systemic medications. Drug-induced retinopathy represents a disease that, although not common, requires early recognition: if not detected early, it may progress and cause irreversible retinal dysfunction with subsequent visual impairment. This review aims to evaluate the use of mfERG in the assessment of retinal dysfunction associated with various systemic pharmacological agents based on the currently available literature. The most commonly recognized systemic medications affecting retinal function are included, such as chloroquine and hydroxychloroquine, vigabatrin, deferoxamine, ethambutol, interferon-α, tamoxifen, digoxin, sildenafil, canthaxanthin, amiodarone and nefazodone. The role of mfERG in the early diagnosis of retinal toxicity and the evaluation of disease severity is reviewed, as well as its clinical value in monitoring disease progression or recovery after drug cessation.