[Melanoma-associated retinopathy with night blindness. Case report]. / Melanom-assoziierte Retinopathie (MAR) mit Nachtblindheit. Fallbericht.

BACKGROUND: Night blindness is usually symptomatic of retinal dysfunction. However, apart from congenital forms of night blindness such as congenital stationary night blindness (CSNB) and pigmentary retinopathy without clumping of pigment, the acquired forms of night blindness present a particular diagnostic challenge to the ophthalmologist. CASE REPORT: A 51-year-old patient with formerly healthy eyes presented with malignant skin melanoma and sudden night blindness. Along with reduced acuity, concentric visual field limitation, and a marked decrease in sensitivity of the retinal rods and cones in adaptometrical tests, significantly reduced b waves and intact a waves were registered in the flash-ERG of both eyes with otherwise inconspicuous morphologic findings. Furthermore, serum levels of antibodies (IgG) against retinal bipolar cells were found to be increased. CONCLUSION: Results indicate the presence of melanoma-associated retinopathy (MAR), which-like carcinoma-associated retinopathy (CAR)-ranks among the tumor-associated diseases of the retina. CAR, MAR, and CSNB can be differentiated immunohistochemically by serum autoantibody determination and electrophysiologically by flash-ERG. As opposed to CAR, the immune response in the case of MAR is not to antigens of photoreceptors and ganglion cells, but to retinal so-called ON-depolarizing bipolar cells mainly connected in series to the rods. In addition, a waves are intact and b waves extinct, resembling the situation of CSNB.

Pupil dilation response to tropicamide: a biological test for Alzheimer's disease?

The pupil dilation response to tropicamide has been proposed as a simple test for demonstrating the central cholinergic deficit present in Alzheimer's disease (AD). We examined this response in 20 patients with clinically diagnosed mild probable AD and in 20 unrelated, cognitively intact, age-matched controls. A marked increase in pupil diameter was observed within 30 minutes in both groups. Differences between the groups did not attain statistical significance. The ability of the proposed test to discriminate between AD patients and healthy controls may be influenced by experimental procedures and by the ethnic background of the subjects under study. At present the pupil dilation response to tropicamide cannot be considered a useful diagnostic aid.

Renal transplantation in 22 children with nephropathic cystinosis.

In 1989, 22 children (11 boys, 11 girls aged 8-23 years) with nephropathic cystinosis, who had received a total of 28 renal allografts over the previous 14 years, were reviewed. Nineteen were alive, of whom 17 had functioning grafts 5 months to 13 years after transplantation. The mean serum creatinine level in these 17 was 135 mumol/l. Patient and graft survival did not differ from non-cystinotic children. Persistent hypothyroidism was found in 3 patients, transient diabetes mellitus in 1, severely disturbed vision in 1 and brain atrophy in 11. Arterial hypertension was present in 16 patients. Growth retardation was universal, although in 4 patients on cyclosporin A post-transplant catch-up growth occurred. Five patients over 15 years completed puberty. Readjustment in terms of school performance was good but was less good for psychosocial development. None of the patients had ever been treated with cystine-depleting agents; the data will therefore provide a historical control group with which to compare the results from a group treated with these agents.

[Tarsus-plasty with chondroplasty]. / Tarsusplastik mit Chondroplast.

For a few years new Chondroplast has been available as a ready-to-use product in a variety of different shapes and sizes. The material is obtained from bovine cartilage and is made antigen-free and stable against decomposition by treatment with glutaraldehyde and irradiation. To date we have used Chondroplast for lid stabilization in 9 eyes with very slack entropion or ectropion. For tarsusplasty secondary to tumor resection we chose Chondroplast for seven eyes. A thin 0.5-mm-thick lamella was cut out of the original material and positioned in a preformed pocket between the orbicularis muscle and the skin and conjunctiva and then fixed with Vicryl sutures. Corrective of eyelid malposition was combined with pentagonal excision for ectropion, or resection of the orbicularis muscle for entropion. In plastic lid replacement the material was sutured to the existing wound edges of the lid margin and covered posteriorly with conjunctiva and anteriorly with advancement or transposed flaps. In all patients the implant took well, and there were no complications during wound healing. The cosmetic and functional results were satisfactory. The largest post-operation follow-up time was over 1 year. In one case--1 year after resection of a lower eyelid basalioma--we had the opportunity to examine histologically the tissue in the area of a Chondroplast implant. We performed this biopsy excision because of a suspected recurrence. Macroscopically the implanted cartilage lamella was surrounded by a barely vascularized capsule. Histologically we found a non-vital cartilage implant surrounded by a mainly fibrotic connective tissue capsule. In only a few places was there evidence of a slight reaction to the foreign body but without substantial inflammation activity. There was also no histological indication of resorption of the implant.

Pattern ERGs and VEP topography evoked by lateral eccentric pattern reversal stimulation.

In ten healthy subjects we recorded simultaneously pattern-ERGs and multichannel VEPs evoked by checkerboard reversal stimuli presented in the center, and at three different locations on the nasal and temporal retina. In the near periphery significantly larger pattern-ERG amplitudes were found for the nasal retina. Farther out in the periphery this effect reversed, and stimuli presented to the temporal retina yielded larger pattern-ERG amplitudes. Component latencies showed also differences with larger latencies for nasal than temporal retinal stimuli in the near periphery. In addition, pattern-ERG amplitudes increased with check size while peak latencies decreased. Retinal stimulus location had a significant influence on the VEP topography reflected by potential distributions lateralized over occipital scalp areas contralateral to the hemiretina stimulated. There were no differences between VEP activity evoked by nasal or temporal retinal stimuli. The results relate to differences in local retinal organization reflected by the distribution of photoreceptors and other neural elements across the retina as well as to functional differences in visual information processing between different retinal areas.

Electrophysiological findings of neuronal ceroid lipofuscinosis in heterozygotes.

Nineteen obligate heterozygotes, 8 individuals at risk of being heterozygote, and 10 patients afflicted with four different forms of neuronal ceroid lipofuscinosis were examined electrophysiologically. The group of obligate heterozygotes was compared to age-matched control groups. Statistically significant differences were found between scotopic b-wave amplitudes, P-ERG amplitudes, and EOG light peaks of the obligate carriers of the juvenile type and the control subjects. The photopic L-ERGs and the latencies of the VEPs were mostly within the normal range. The findings represent the first evidence of functional ophthalmological changes in obligate carriers of neuronal ceroid lipofuscinosis and demonstrate that heterozygotes with certain hereditary autosomal recessive diseases may manifest subtle functional signs.

Pattern electroretinogram: effects of miosis, accommodation, and defocus.

The effect of miosis, lens accommodation, and defocusing on the various components of the pattern electroretinogram elicited by checkerboard reversal was investigated by employing aperture stops of different size, varying the mean luminance of the target, and inserting spherical lenses of various optical diopters in front of the eye. After topical administration of pilocarpine (1%) the following changes in the pattern electroretinogram were observed: (i) a decrease of the pupillary diameter leading to a lowered target luminance, which produced attenuation of PERG amplitudes and prolonged latencies, and (ii) defocusing by accommodation of the crystalline lens, which produced attenuation of PERG amplitudes no effect on latencies.

[Mastopathy and breast cancer: is there a typical hormone profile?]. / Mastopathie und Mammakarzinom: Besteht ein typisches Hormonprofil?

Carcinoma of the breast and mastopathy are statistically correlated with the functioning of the gonads. The authors studied whether in 58 women with carcinoma of the breast and in 36 patients with mastopathy any change was seen in the secretory pattern of gonadal steroids and whether there were any latent changes in the secretion of prolactin and TSH. A group of 43 women with benign gynaecological diseases served as controls. Before making the diagnosis via immediate section for microscopic examination we determined the basal values of prolactin, LH, FSH, TSH and the oestrogen fractions E1, E2, E3, total oestrogens and progesterone. In addition, we measured the increase in prolactin and TSH after stimulation with metoclopramide. Postoperatively this was followed by measuring the basal values of prolactin and TSH and their secretory performance in respect of TRH. All histological diagnoses were made by an experienced pathologist. Examinations were performed under strictly standardised conditions, taking into account stress, circadian variation and the nutritional status. The following group-specific significant results were obtained (p less than 0.05-0.01): Mastopathy: high serum values of all oestrogen fractions and of total oestrogens, high PRL release through MTC stimulation, prolactin secretory effect after MTC in accordance with the degree of severity of epithelial proliferation, early onset of menopause. Carcinoma of the breast: Low PRL and TSH release on MTC stimulation, low progesterone concentrations during the second cycle phase, relatively high serum concentrations of oestradiol during the first cycle phase, PRL basal values increased postoperatively, compared with preoperative values. The results led to the following conclusions: In mastopathy patients there is a connection between high oestrogen concentrations and consecutively high prolactin release. The morphological substrate is represented by an enhanced proliferation of epithelium. In patients with cancer of the breast reduced progesterone concentrations during the second half of the cycle lead us to suspect an increased incidence of luteal insufficiency; in addition, it appears that the daily prolactin secretion rate in this group is on a reduced level.