Biomedical subjects
Publications and source records attributed to E Leide.
The combination of retinopathia pigmentosa and Coats syndrome is described. The clinical picture shows spotty pigmentations, retinal neovascularizations, peripheral exudative retinal detachment, defects of the retinal pigment epithelium, and changes in the macula.
Sixty-six non-preselected children admitted to Düsseldorf University Clinic with head injuries were examined ophthalmologically (O) and neurologically (N) in the course of a preliminary study. The severity of the injury was classified according to Lange-Cosack the injury was classified according to a proposal of Lange-Cosack and Tepfer (I-VI). The following numbers of patients presented with symptoms: I: O + N = 6 (out of 24); N = 6 (24); O = (24) II: O + N = 4 (11); N = 4 (11); O = 1 (11) III: O + N = 6 (11); N = 2 (11); O = 1 (11) IV: O + N = 5 (5) V: O + N = 7 (7) VI: O + N = 8 (8) Nystagmus was observed in 13 cases, a pathologic response of the pupil in 11 cases, motility disturbances in 11 cases, convergence disorders in nine cases and injury of the orbit in six cases. The visual evoked cortical response (VEP) was pathologic in 23 patients while there were pathologic conditions at the fundus in eight cases and visual field defects in three. A disorder of the VEP was thus the most common finding in this study.
A comparison between the fundus photographs and the fluorescein angiogram at the beginning of this disease shows the great informative value of fluorescein angiography. At first the distinct functional losses are most important; ophthalmoscopically, only slight pigment irregularities are discernible. In the fluorescein angiogram the picture of fundus flavimaculatus is already distinguishable at this stage of the disease. These findings led support to the view that Stargardt's macular degeneration and fundus flavimaculatus are identical.
In a 74-year-old patient with sudden loss of vision the ERG was normal while the VEPs were highly pathologic. CT confirmed a bilateral vascular lesion in the perfusion area of the posterior cerebral arteries.
By means of fluorescein angiography extensive defects in the pigmented epithelium found in choroideremia can be shown to be present in the first ten years of life. The degeneration of the vessels of the choroid and the functional damage to the retina do not seem to take place until later.
Changes in the macula in Retinitis pigmentosa, especially the "Bull's Eye" macula, are shown with fluorescein angiograms. Functional tests complement the morphological findings.
The fluorescein angiogram makes a widespread degeneration of the retinal pigment epithelium in choroidermia visible. In the female carriers it shows a spot-like picture. This method of examination contributes to the interpretation of the pathogeny and differential diagnosis.
A 38-year-old patient showed monocular nonprogressing pigmentary changes for 8 years, with nearly normal blood vessels and scarcely restricted dark-adaptation. ERG and VER were different from those of the other eye. The affected eye had full vision and an annular scotoma corresponding to the pigmentary changes. The other eye was amblyopic due to strabismus. Phenotypes, similar to retinitis pigmentosa, and chorioretinitis diffusa are discussed. This is probably a congenital anomaly.
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