Normal Electroretinography Research Articles

Electroretinographic findings in Duchenne/Becker muscular dystrophy and correlation with genotype

Purpose: To correlate the electroretinography (ERG) findings in patients with Duchenne/Becker muscular dystrophy (D/BMD) with the genotype and evaluate the ERG findings in DMD carriers. Materials and methods: Fifteen deletion positive patients with DMD, two deletion positive patients with BMD and six DMD carriers in two families having a positive disease history were evaluated with DNA analysis, ophthalmologic and ERG findings. Results: The mean age of the deletion positive D/BMD patients was 11.1 ± 3.7 (range: 7–20).No abnormality was detected in the ophthalmic examination of the patients. In three out of 17 patients with D/BMD, ERG was normal whereas in 14 patients electronegative ERG was recorded in scotopic conditions. Mutational alterations observed in these three cases with normal ERG were located exceptionally at 5' hot spot; not like the others within central region. In all DMD carriers ERG findings were recorded as normal. Conclusion: ERG findings in D/BMD patients may show some correlations with molecular analysis, whereas it is not useful in DMD carriers.

Inhibition of diclofenac formulated in hyaluronan on angiogenesis in vitro and its intraocular tolerance in the rabbit eye.

To investigate the effect of diclofenac, a potent nonsteroidal anti-inflammatory drug, formulated in hyaluronan (diclofenac/HA) on angiogenesis in vitro and its intraocular toxicity in vivo. The effect of diclofenac/HA on angiogenesis was determined by choriocapillary endothelial cells on Matrigel stimulated by vascular endothelial growth factor (VEGF). The tube areas were quantified by image digital analysis. For toxicity study, diclofenac/HA was injected intravitreally with a dose range from 100 to 1080 microg in 26 rabbits following gas compression vitrectomy. Potential toxicity was assessed by indirect ophthalmoscopy and by histological studies (light and electron microscopy). Retinal function was monitored by electroretinography (ERG) in six rabbits that received 400 microg of diclofenac/HA. Diclofenac/HA, 180, 90 microg/ml, inhibited tube formation to 24% and 55% of the standard group (Media Ham's F12 plus 5% fetal calf serum and 50 ng/ml VEGF) respectively (P<0.01). Intravitreal injection of 540 microg or higher doses of diclofenac/HA resulted in ocular toxicity in the rabbit, demonstrated as cataract, vitreous haze and retinal damage observed by indirect ophthalmoscopy and light- and electron-microscopic examinations. No toxicity was observed in the eyes that received 400 microg or less diclofenac/HA, which was further supported by the normal ERG examined at 4 and 25 days post injection. Diclofenac/HA inhibits tube formation in vitro and is non-toxic to the rabbit retina at concentrations that are inhibitory to tube formation. Our results suggest diclofenac/HA may be an effective candidate to inhibit ocular neovascularisation related to granulomatous reaction in the eye.

Electrophysiology of type II mesangiocapillary glomerulonephritis with associated fundus abnormalities.

The retinal electrophysiology is reported in four patients with type II mesangiocapillary glomerulonephritis and partial lipodystrophy with associated fundus abnormalities and no visual symptoms. The histological hallmark of the condition is that of widespread electron dense deposits in the renal glomerulus and in the choriocapillaris and Bruch's membrane of the eye. Three of the four patients had the typical fundal appearance of multiple, yellow, drusen-like lesions at the posterior pole of the eye with normal visual acuity. These three patients had abnormally low Arden ratios on electro-oculography with normal electroretinography responses. This is the first clinical model of disease known to be isolated to the choriocapillaris and Bruch's membrane causing an electro-oculographic abnormality without any clinically detectable deficit in visual function.