Solar retinopathy. A study from Nepal and from Germany.

319 patients with a solar retinopathy were seen in an eye clinic in Nepal within 20 months. All patients had either a positive history of sun-gazing or typical circumscribed scars in the foveal area. In more than 80% of the patients the visual acuity was 6/12 or better and did not deteriorate over time. 126 (40%) patients had a history of gazing at the sun during an eclipse, 33 (10%) were sun worshipers and 4 (1%) were in both categories. Three years later 29 patients were re-examined in a follow-up study. Only 16 had had visual disturbances directly after they had gazed into the sun. No colour vision defects were seen in any of the 44 affected eyes, when tested with Panel D 15, while four patients (6 eyes) had some uncertainty with the tritan plates of the Ishihara test charts. Metamorphopsia were recorded in 11 eyes. Five German patients with solar retinopathy were examined in more detail. Colour contrast sensitivity (CCS) was tested for the central and the peripheral visual field. CCS for tritan axis was raised in all patients for the central visual field, while it was normal for the peripheral visual field.

[Color vision defect. A early sign of Sorsby retinal dystrophy?]. / Farbsinnstörung. Ein Frühzeichen bei Sorsby-Netzhautdystrophie?

In 34 asymptomatic patients from three families at 50% risk of developing Sorsby's fundus dystrophy, colour contrast sensitivity was measured. In 16 the thresholds--mainly of the tritan axis--were raised above the normal values. It is concluded that testing of colour vision is useful in detecting the abnormal genotype. Raised colour contrast sensitivity was not observed before the first fundus or fluorescein angiographic changes in two of the families, while the colour defect occurred in the absence of ophthalmoscopic abnormality in the third family. Therefore, our results support the clinical suggestion that Sorsby's fundus dystrophy is more than one disease.

Detection of the G to A mitochondrial DNA mutation at position 11778 in German families with Leber's hereditary optic neuropathy.

Leber's hereditary optic neuropathy (LHON) is characterized by acute or subacute bilateral (usually permanent) loss of central vision, caused by neuroretinal degeneration. The maternal inheritance is explained by the mitochondrial origin of the disease. Recently, a single mitochondrial DNA (mtDNA) mutation, a G to A substitution at position 11778 that converts a highly conserved arginine to histidine, has been associated with LHON. The mutation eliminates an SfaNI restriction enzyme recognition site and thus provides a method for detection of the mutation by amplification, enzyme digestion and agarose gel electropheresis of polymerase chain reaction (PCR) products. Leukocyte mtDNA from 7 German families with LHON, diagnosed by clinical criteria, was tested for the presence of the G to A mutation at bp 11778. The mtDNa mutation, detected as a loss of the SfaNI site, was seen in one family. The G to A mtDNA mutation is the only known gene alteration associated with LHON so far. It has been identified in patients of different ethnic origin and recent reports strongly support the hypothesis that it represents the most frequent cause of LHON. Identification of the mtDNA replacement mutation using PCR and restriction enzyme digestion requires only a small amount of blood and can be performed rapidly. This method is thus a useful tool in the diagnosis of LHON.

Colour contrast sensitivity changes caused by peripheral retinal laser photocoagulation.

Macular phototoxicity is known to occur with laser use, and there is evidence that the wavelength of the light used influences this effect. In this study, a computer based colour contrast sensitivity test was used to assess the immediate macular effects of photocoagulation of peripheral flat retinal holes in otherwise normal retinas, using blue-green (488 and 514 nm), yellow (577 nm), orange (595 nm) or red (647 nm) laser light. The laser aiming beam was not allowed to traverse the macula at any stage during treatment. No protan or deutan axis threshold changes were noted in the 17 patients tested irrespective of the laser wavelength used. Tritan axis sensitivity was significantly reduced one hour after treatment with the blue-green laser, but no tritan axis change was found after treatment with longer wavelength lasers. The effect was no longer present the day after treatment in the subjects tested. The results show that even peripheral retinal treatment with blue-green laser can cause acute macular phototoxicity.

Electrophysiology and colour perimetry in dominant infantile optic atrophy.

A typical finding in dominant infantile optic atrophy (DIOA) is the variation of the phenotypic expression of the DIOA gene even within one family. It is of special interest for genetic consultation to evaluate an examination method for detecting subclinically involved patients. Seven patients of two families were examined. Three of them had the typical symptoms of DIOA: reduced visual acuity, tritan defect, temporal pallor of both optic discs, and a relative central scotoma for white test spots. In visual evoked cortical potentials (VECP) the amplitudes were reduced, and in one patient the latencies were slightly delayed and two patients considerably so. The amplitude of the negative component of the PERG was markedly reduced, while the positive component was normal. In the remaining four family members normal retinal and cortical responses were recorded under standard conditions and visual fields and colour vision (FM 100 hue) were also normal. However, static perimetry with blue test spots showed in two family members enlarged central scotomas, thus proving that they had subclinical DIOA.

[Electro-ophthalmologic studies in HIV patients]. / Elektroophthalmologische Untersuchungen bei HIV-Patienten.

We examined 44 HIV-positive patients in different disease stages with electroretinogram (ERG), pattern-electroretinogram (PERG), and visually evoked cortical potentials (VECP). Sixty-eight of the 88 eyes examined had a normal fundus and full central vision. Twelve eyes showed cotton-wool ecudates and 8 eyes CMV retinitis. Fifty-four eyes with normal fundus were examined by ERG. Of these 28 (52%) showed marked reduction of the amplitude. In the PERG, 20 eyes out of 50 examined (40%) showed an amplitude reduction. In the VECP, 12 out of 65 eyes (19%) had a reduced amplitude. In the ERG, 7 of 11 eyes (64%) with cotton-wool exudates showed marked pathological findings, as opposed to 4 of 10 cases (40%) in the PERG and 3 of 12 (12%) in the VECP. Seventy-five percent of the eyes with CMV retinitis (6 of 8 cases) showed pathological findings in the ERG and VECP and 100% (all 7 cases examined) in the PERG. These electrophysiological findings suggest that there are diffuse disorders in the retina of HIV-positive patients. It is possible that these findings are based on direct infection of the retina with HIV, or that they represent a vascular disorder, subclinical infection or are related to side effects of the drugs used for the HIV infection.

[Color vision disturbance in laser operators and patients: comparison of argon and dye lasers]. / Farbsinnstörungen bei Laser-Operateuren und Patienten: Ein Vergleich zwischen Argon- und Dye-Laser.

Color contrast sensitivity was measured in laser operators before and after laser sessions. The tritan threshold was elevated after the sessions in all laser operators who had used argon (blue-green) lasers. No change was observed when lasers with longer wave-lengths were used. Elevation of the tritan threshold to above-normal levels was observed even before laser sessions. The thresholds remained increased even when the laser operator did not use a laser for 3 weeks. Thus, it appears that the damage caused by the argon blue-green laser is cumulative. Elevated tritan thresholds were also observed in patients with peripheral retinal breaks who were treated with an argon (blue-green) laser.

Pattern electroretinograms in optic neuritis during the acute stage and after remission.

A total of 20 patients with unilateral acute optic neuritis were studied. Each patient had experienced the recent onset of a decrease in visual acuity, a relative afferent pupillary defect, a relative or absolute central scotoma and a colour-vision defect. The pattern-reversal electroretinogram (PERG) of each patient was analysed with regard to the amplitude of the positive and negative components. During the acute stage the amplitude of the positive component was reduced in all patients and that of the negative, in 18 of 20 cases. Parallel to clinical recovery, a steady increase was observed in the amplitude of the positive component to normal values; no statistical differences between affected and fellow eyes was found. In contrast, the amplitude of the negative component remained significantly reduced after clinical recovery.

Using argon laser blue light reduces ophthalmologists' color contrast sensitivity. Argon blue and surgeons' vision.

Color contrast sensitivity was measured in laser operators before and after laser use. After argon blue-green laser treatment sessions, sensitivity was reduced for colors lying along a tritan color-confusion line for several hours. This acute effect is due to specular "flash-backs" from the aiming beam off the surface of the contact lens. It is caused only by argon 488-nm light, when the aiming beam intensity is high. In addition, a correlation has been demonstrated between the number of years of laser experience and a chronic reduction in tritan color contrast sensitivity. It is suggested that repeated acute changes caused by the argon lasers may cause cumulative effects and produce a chronic threshold elevation. A simple method of eliminating the acute effect is documented.

Leber's hereditary optic atrophy.

Leber described a particular type of hereditary optic atrophy in 1871. The clinical features of all cases since reported are reviewed. There is characteristically acute visual loss, circumpapillary teleangiectatic micropathy, tortuosity of the retinal vessels and oedema in the retinal nerve fibres. 85% of those affected are male, but affected fathers do not transmit the condition to their children. The exact mode of inheritance is still obscure but it is suggested that inheritance may be mitochondrial; enlarged subsarcolemmal mitochondria in LOA patients have been described. Colour vision defects are observed not only in patients, but also in presumed carriers. Electrophysiological investigations demonstrate optic nerve damage, but are not indicative of any particular pathology. It has been reported that in many cases of LOA the severity of the disease is related to tobacco smoking. Increased cyanocobalamin blood levels in patients and increased cyanide blood levels support this hypothesis.

Separable evoked retinal and cortical potentials from each major visual pathway: preliminary results.

Single cell experiments in primates show that there are two major parallel pathways named after the lamination in the lateral geniculate nucleus. Each of these systems can be preferentially excited by appropriate stimuli. Here we report that in man the polarity of the evoked potentials both in retina and in cortex depends on which of these pathways is stimulated. The identification of the resulting waveforms is thereby simplified--a matter of practical importance. The fact that at retina and cortex there are characteristic potentials may reflect the different cell biology of the two pathways.

Leber's hereditary optic atrophy: further evidence for a defect of cyanide metabolism?

We studied one patient with Leber's optic atrophy (LOA) in the acute stage and 12 at the chronic stage of the disease, and measured the activity of rhodanese in white blood cells and the level of cyanide in whole blood. In the patient with acute disease the blood cyanide level was significantly increased at first. Treatment of this patient with cyanide antagonists reduced his cyanide level, but this was not accompanied by improvement in visual function. Rhodanese activity was normal in all patients, as were the blood cyanide levels in each of the 12 patients at the chronic stage of the disease. These findings suggest a temporary disturbance of cyanide metabolism during the acute phase of the disease in this family. The abnormal metabolic mechanism was not identified.

Electrophysiology and perimetry in acute retrobulbar neuritis.

Eighteen patients with acute retrobulbar neuritis were examined by visual field analysis and electroophthalmological recordings. The visual fields were measured to 30 degrees with the automatic perimeter Octopus 201. The pattern-evoked cortical potential showed an increased P-100 latency followed by a decrease in 12 patients during the first two weeks of the disease. This latency change cannot be explained by remyelinization. Teh positive component of the pattern ERG (P-50) was reduced in all acutely affected eyes in comparison with the fellow eye. During recovery, the amplitude of the positive PERG component increased to normal, but remained slightly reduced in comparison with the response from the fellow eyes. Thirteen patients out of 18 were clinically and electrophysiologically diagnosed as having unilateral retrobulbar neuritis. However, static perimetry showed paracentral scotomas in the fellow eye in 5 cases. Recovery of the severely affected eye was accompanied by complete normalization of the visual field in the fellow eye. Thus static perimetry seems to be a more sensitive test than even the visual evoked potentials for detecting lesions in the visual pathway.

The pattern electroretinogram.

Physiological experiments and the exploitation of clinical conditions have provided compelling evidence that retinal ganglion cells and other inner retinal structures generate the pattern ERG (PERG). As an increasing number of clinical reports have been published some contradictory findings have been reported. These may be ascribed to variation in recording and measuring techniques. The PERG consists of two major portions, the early positive and the following negative component which can be investigated separately if the stimulus conditions allow isolated (or "transient") responses to be recorded. Care has to be taken in positioning the reference electrode, maintaining accurate refraction, and the influence of pupil size must be considered. Furthermore the PERG is contaminated by a luminance component which may be generated in the outer retina. The size of this increases with low spatial frequency (large check-sizes) and high mean luminance. The PERG permits the examination of an additional level of the retina and helps the understanding of pathophysiology of various eye diseases, and is of clinical importance in routine diagnosis and assessment. In glaucoma the PERG amplitude is often reduced before it is possible to detect a scotoma and it is therefore an important prognostic indicator in patients with ocular hypertension. In diabetic retinopathy, retinal ischaemia sufficient to lead to the pre-proliferative state can be demonstrated. The PERG also has a major clinical role in examining localised retinal pathology. If combined with VECP recording, it greatly extends the interpretations possible, since not only can damage to the optic nerve be detected by both tests, but the normal PERG in the presence of an abnormal PVECP implies that the losses are confined to the central pathway.