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Biomedical subjects

J G Notting

Publications and source records attributed to J G Notting.

7 recordsLinked to original sources

[Dominant cystoid macular dystrophy (author's transl)].

Dominant cystoid macular dystrophy (D.C.M.D.) is characterized by a macular dystrophy but at the same time by a pigmentary dystrophy of the retinal periphery. Ultimately D.C.M.D. resembles an atypical pigmentary dystrophy, in some cases that of a pericentral retinitis pigmentosa. In an early stage the results of the EOG and darkadaptation curve reflect the process at the level of the peripheral retina, while only in a late stage we may expect some diffuse ERG pathology. D.C.M.D. might be classified as a tapetoretinal dystrophy and in particular as a form of retinitis pigmentosa with an atypical visual disturbance and an atypical fundus appearance (Leber, 1871). In doing so attention is paid to the fact that D.C.M.D. is more than a macular dystrophy. The denomination of the disease as D.C.M.D. reflects the most important features at a relatively young age.

Diagnosis, Differential↗

Dominant cystoid macular dystrophy.

Of 24 examined family members, 14 had an undescribed macular dystrophy. There is a clear autosomal-dominant heredity. Essential features of the disease are pigmentary disturbance, cystoid macular edema, wrinkling of the internal limiting membrane, and vitreous body changes. In this family there was an increased incidence of severe hyperopia (greater than or equal to 6 diopters). As the disease progressed color vision deteriorated (type 1 acquired red-green defect), disturbance of the electro-oculogram became more prevalent, and the electroretinogram was normal. These finding differentiate this dystrophy from other diseases with a bull's eye aspect or cystoid macular edema and from vitreoretinal syndromes.

Aged↗

Retinal functions in dominant cystoid macular dystrophy (DCMD).

Dominant cystoid macular dystrophy (DCMD) occurred in 28 members of 5 unrelated families. The disease is characterized by cystoid macular oedema and leakage from retinal capillaries in the posterior pole. Colour vision examination reveals a type I red-green defect with concomitant blue-yellow defectiveness; the latter may be caused by the leaking capillaries. The ERG is normal. The EOG is subnormal. Darkadaptation curves are often slightly disturbed. There are frequently also aspecific pigmentary alterations in the peripheral fundus.

Color Perception↗

[Diagnosis and treatment of macular lesions in multifocal chorioiditis (presumed histoplasmosis) (author's transl)].

The authors describe the clinical picture of "presumed histoplasmosis" with the typical disciform mascular lesion which is found in a increasing number of Dutch patients. Although systemic histoplasmosis is not endemic in the Netherlands, the authors feel they are dealing with the same entity. The therapy is elucidated, especially (Argon laser) photocoagulation of favourably situated subretinal neovascularisations. The importance of adequate coagulation technique and frequent (fluographic) follow-up studies in view of possible complications is stressed.

Adult↗