Flicker Fusion Thresholds in Best Macular Dystrophy

Critical Flicker Frequency as a Potential Vision Technique in the Presence of Cataracts

“…In fact, the CFF technique showed an excellent sensitivity for identifying normal visual function and hence for predicting successful postoperative outcome, with a 97% accuracy in the nonsurgical cataract sample. In agreement with previous studies, 21,36,37 the CFF technique also appeared to be sensitive to the presence of MD. Although different mechanisms mediate the appreciation of VA (spatial processing) and the detection of CFF (temporal processing), 38 a significant association between VA and CFF was found for results from the MD group (Fig.…”

Critical Flicker Frequency as a Potential Vision Technique in the Presence of Cataracts

“…In fact, the CFF technique showed an excellent sensitivity for identifying normal visual function and hence for predicting successful postoperative outcome, with a 97% accuracy in the nonsurgical cataract sample. In agreement with previous studies, 21,36,37 the CFF technique also appeared to be sensitive to the presence of MD. Although different mechanisms mediate the appreciation of VA (spatial processing) and the detection of CFF (temporal processing), 38 a significant association between VA and CFF was found for results from the MD group (Fig.…”

A pathogenic in-frame deletion-insertion variant in BEST1 phenocopies Stargardt disease

“…This patient was also found to have the same heterozygous variant in BEST1. EOG, although affected by the patient's poor fixation, showed diminished light rise bilaterally and ffERG revealed preserved scotopic rod-specific responses with a low B to A ratio, suggesting inner retinal dysfunction consistent with published literature (21)(22)(23), and diminished photopic-cone single flash and 30Hz flicker responses. While ffERG findings are often normal in early BVMD and Stargardt disease, diminished scotopic and photopic responses can be expected in later stage Stargardt disease (20).…”

The pattern ERG in Best's disease

“…These findings have led them to believe that the sensory retina may be the primary seat of the disease process. As far back as 1977 Massof demonstrated abnormal flicker fusion thresholds in a group of twenty five patients with Best's disease and normal vision [5]. They concluded that the disease affects the neurosensory retina, even when Snellen visual acuity was normal.…”

Critical Flicker Frequency as a Potential Vision Technique in the Presence of Cataracts

“…In fact, the CFF technique showed an excellent sensitivity for identifying normal visual function and hence for predicting successful postoperative outcome, with a 97% accuracy in the nonsurgical cataract sample. In agreement with previous studies, 21,36,37 the CFF technique also appeared to be sensitive to the presence of MD. Although different mechanisms mediate the appreciation of VA (spatial processing) and the detection of CFF (temporal processing), 38 a significant association between VA and CFF was found for results from the MD group (Fig.…”

A pathogenic in-frame deletion-insertion variant in BEST1 phenocopies Stargardt disease

“…This patient was also found to have the same heterozygous variant in BEST1. EOG, although affected by the patient's poor fixation, showed diminished light rise bilaterally and ffERG revealed preserved scotopic rod-specific responses with a low B to A ratio, suggesting inner retinal dysfunction consistent with published literature (21)(22)(23), and diminished photopic-cone single flash and 30Hz flicker responses. While ffERG findings are often normal in early BVMD and Stargardt disease, diminished scotopic and photopic responses can be expected in later stage Stargardt disease (20).…”

The pattern ERG in Best's disease

“…These findings have led them to believe that the sensory retina may be the primary seat of the disease process. As far back as 1977 Massof demonstrated abnormal flicker fusion thresholds in a group of twenty five patients with Best's disease and normal vision [5]. They concluded that the disease affects the neurosensory retina, even when Snellen visual acuity was normal.…”

Critical Flicker Frequency as a Potential Vision Technique in the Presence of Cataracts

“…In fact, the CFF technique showed an excellent sensitivity for identifying normal visual function and hence for predicting successful postoperative outcome, with a 97% accuracy in the nonsurgical cataract sample. In agreement with previous studies, 21,36,37 the CFF technique also appeared to be sensitive to the presence of MD. Although different mechanisms mediate the appreciation of VA (spatial processing) and the detection of CFF (temporal processing), 38 a significant association between VA and CFF was found for results from the MD group (Fig.…”

A pathogenic in-frame deletion-insertion variant in BEST1 phenocopies Stargardt disease

“…This patient was also found to have the same heterozygous variant in BEST1. EOG, although affected by the patient's poor fixation, showed diminished light rise bilaterally and ffERG revealed preserved scotopic rod-specific responses with a low B to A ratio, suggesting inner retinal dysfunction consistent with published literature (21)(22)(23), and diminished photopic-cone single flash and 30Hz flicker responses. While ffERG findings are often normal in early BVMD and Stargardt disease, diminished scotopic and photopic responses can be expected in later stage Stargardt disease (20).…”

The pattern ERG in Best's disease

“…These findings have led them to believe that the sensory retina may be the primary seat of the disease process. As far back as 1977 Massof demonstrated abnormal flicker fusion thresholds in a group of twenty five patients with Best's disease and normal vision [5]. They concluded that the disease affects the neurosensory retina, even when Snellen visual acuity was normal.…”