RESUMO
We studied visual function in 16 patients with the Bardet-Biedl syndrome. Visual acuity, kinetic perimetry, and electroretinography results indicated a severe loss of central and peripheral vision and rod and cone function by the second or third decade of life. Light- and dark-adapted static perimetry in patients 10 to 15 years of age with early involvement showed a parallel and marked loss of rod and cone sensitivity across the visual field. Patients with more advanced disease and no measurable peripheral visual field showed different patterns of central visual dysfunction: an island of only cone function centered in a bull's-eye lesion; patches of rod function surrounding geographic atrophy; or a central island of excellent rod sensitivity but severely impaired cones. In the two least-affected patients, a 13-year-old boy and the asymptomatic 45-year-old sibling of a patient, there were more rod than cone abnormalities as determined by electroretinography and static perimetry.
Assuntos
Síndrome de Laurence-Moon/fisiopatologia , Células Fotorreceptoras/fisiopatologia , Adolescente , Adulto , Criança , Sensibilidades de Contraste , Adaptação à Escuridão , Eletrorretinografia , Feminino , Fundo de Olho , Humanos , Masculino , Retinose Pigmentar/fisiopatologia , Acuidade Visual , Testes de Campo Visual , Campos VisuaisRESUMO
Methods have been developed to compare full field rod and cone electroretinograms with results of rod and cone static perimetric measurements across the visual field. In a limited number of patients with retinal degeneration, including two subtypes of retinitis pigmentosa, there were close relationships between electroretinographic and psychophysical parameters. Maximum b-wave amplitude and visual field area were highly correlated, as were electroretinographic and perimetric measures of sensitivity loss. Future application of the methods to large numbers of patients with typical retinitis pigmentosa may help elucidate different mechanisms of retinal degeneration.
Assuntos
Eletrorretinografia , Células Fotorreceptoras/fisiopatologia , Psicofísica/métodos , Degeneração Retiniana/fisiopatologia , Adulto , Idoso , Criança , Adaptação à Escuridão , Feminino , Humanos , Masculino , Modelos Biológicos , Testes de Campo VisualRESUMO
Visual fields and dark-adapted thresholds, essential measurements of visual function in patients with retinitis pigmentosa (RP), are usually performed manually. The authors have modified a computerized perimeter to perform automated light- and dark-adapted static perimetry across the visual field of RP patients. Results permit assessment of the level of visual disability in the light and dark and also help define subtypes of RP.
Assuntos
Adaptação Fisiológica , Adaptação à Escuridão , Luz , Retinose Pigmentar/fisiopatologia , Testes de Campo Visual/métodos , Adolescente , Adulto , Automação , Criança , Computadores , Feminino , Humanos , Masculino , Limiar Sensorial , Testes de Campo Visual/instrumentaçãoRESUMO
Two patients presented with unusual uniocular electroretinographic (ERG) phenomena. One patient showed the ERG characteristics of stationary night blindness in one eye only and the other patient presented with ERG evidence of uniocular cone dysfunction. In both patients, however, further electrophysiological and psychophysical studies and serial testing provided evidence for dysfunction of both receptor systems and relatively subtle involvement of the fellow eyes.
