Fluorescein angiography of the hereditary choroidal dystrophies.

Full‐field electroretinograms in patients with central areolar choroidal dystrophy

“…In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989). In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989).…”

“…In the present study we evaluated the results from full-field electroretinograms in 19 patients with central areolar choroidal dystrophy in order to study the diagnostic and the prognostic value of this examination form. In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989). However, these observations were not statistically analyzed, and it has remained undecided if' there is a systematic decrease in patients with central areolar dystrophy.…”

Full‐field electroretinograms in patients with central areolar choroidal dystrophy

“…In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989). In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989).…”

“…In the present study we evaluated the results from full-field electroretinograms in 19 patients with central areolar choroidal dystrophy in order to study the diagnostic and the prognostic value of this examination form. In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989). However, these observations were not statistically analyzed, and it has remained undecided if' there is a systematic decrease in patients with central areolar dystrophy.…”

Paramacular Choriocapillaris Atrophy

“…sclerosis" to describe a case that was comparable in 1938. Both Schocket and Ballin (1970) and Krill and Archer (1971) describe later cases [4,5]. Assuming an autosomal dominant and autosomal recessive pa ern of inheritance, the genetic pa ern is unknown.…”

“…Although atrophy of the choriocapillary layer is considered to be the crucial event in the regional choroidal dystrophies in the early described cases, early ophthalmoscopic signs of the disease at the level of the RPE, such as patches of hyperpigmentation and depigmentation, were described even back then [4,5]. The presence of hyperautofluorescence in the fovea on FAF images may indicate RPE activity, whereas several indicators of hyper/hypoautofluorescence along the margin of the atrophic and seemingly intact retina may indicate disease progression.…”

Choroideremia in a woman with ectodermal dysplasia and complex translocations involving chromosomes X, 1, and 3

“…Consistent with the findings of Noble et al, fluorescein angiography (FA) confirmed the diffuse choriodal atrophy and defined the area of spared RPE and choroid centrally (Figure 4). 4 Goldmann visual fields demonstrated less than 10-degree central islands in both eyes. Electro-physiologic testing revealed a flat scotopic ERG with small flicker response.…”

Full‐field electroretinograms in patients with central areolar choroidal dystrophy

“…In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989). In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989).…”

“…In the present study we evaluated the results from full-field electroretinograms in 19 patients with central areolar choroidal dystrophy in order to study the diagnostic and the prognostic value of this examination form. In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989). However, these observations were not statistically analyzed, and it has remained undecided if' there is a systematic decrease in patients with central areolar dystrophy.…”

Paramacular Choriocapillaris Atrophy

“…sclerosis" to describe a case that was comparable in 1938. Both Schocket and Ballin (1970) and Krill and Archer (1971) describe later cases [4,5]. Assuming an autosomal dominant and autosomal recessive pa ern of inheritance, the genetic pa ern is unknown.…”

“…Although atrophy of the choriocapillary layer is considered to be the crucial event in the regional choroidal dystrophies in the early described cases, early ophthalmoscopic signs of the disease at the level of the RPE, such as patches of hyperpigmentation and depigmentation, were described even back then [4,5]. The presence of hyperautofluorescence in the fovea on FAF images may indicate RPE activity, whereas several indicators of hyper/hypoautofluorescence along the margin of the atrophic and seemingly intact retina may indicate disease progression.…”

Choroideremia in a woman with ectodermal dysplasia and complex translocations involving chromosomes X, 1, and 3

“…Consistent with the findings of Noble et al, fluorescein angiography (FA) confirmed the diffuse choriodal atrophy and defined the area of spared RPE and choroid centrally (Figure 4). 4 Goldmann visual fields demonstrated less than 10-degree central islands in both eyes. Electro-physiologic testing revealed a flat scotopic ERG with small flicker response.…”

Full‐field electroretinograms in patients with central areolar choroidal dystrophy

“…In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989). In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989).…”

“…In the present study we evaluated the results from full-field electroretinograms in 19 patients with central areolar choroidal dystrophy in order to study the diagnostic and the prognostic value of this examination form. In a few patients some authors have previously found a decrease of the b-wave amplitude in the electroretinogram (Krill & Archer 1971;Noble et al 1977;Weber et al 1985;Castier et al 1989). However, these observations were not statistically analyzed, and it has remained undecided if' there is a systematic decrease in patients with central areolar dystrophy.…”

Paramacular Choriocapillaris Atrophy

“…sclerosis" to describe a case that was comparable in 1938. Both Schocket and Ballin (1970) and Krill and Archer (1971) describe later cases [4,5]. Assuming an autosomal dominant and autosomal recessive pa ern of inheritance, the genetic pa ern is unknown.…”

“…Although atrophy of the choriocapillary layer is considered to be the crucial event in the regional choroidal dystrophies in the early described cases, early ophthalmoscopic signs of the disease at the level of the RPE, such as patches of hyperpigmentation and depigmentation, were described even back then [4,5]. The presence of hyperautofluorescence in the fovea on FAF images may indicate RPE activity, whereas several indicators of hyper/hypoautofluorescence along the margin of the atrophic and seemingly intact retina may indicate disease progression.…”

Choroideremia in a woman with ectodermal dysplasia and complex translocations involving chromosomes X, 1, and 3

“…Consistent with the findings of Noble et al, fluorescein angiography (FA) confirmed the diffuse choriodal atrophy and defined the area of spared RPE and choroid centrally (Figure 4). 4 Goldmann visual fields demonstrated less than 10-degree central islands in both eyes. Electro-physiologic testing revealed a flat scotopic ERG with small flicker response.…”