Suppression scotomas in primary microstrabismus — a perimetric artefact

Static perimetry with stereoscopic targets, stereo-perimetry, was performed on eight patients with primary microstrabismus to find out how strabismic subjects see under natural conditions, i.e. how they ordinarily make use of their squinting eye. In all cases, suppression scotomas were detectable using dissociating perimetric techniques. By means of stereoperimetry, however, the scotomas were not detectable. On the contrary, stereo-acuity was always best in the center of the suppression scotomas. This result indicates that suppression scotomas of the microstrabismic subjects examined here represented perimetric artefacts. In microstrabismic pateints, the cooperation of the deviated eye in the central visual field is probably much better than has previously been thought.     

Melanotic Neuroectodermal Tumor of Infancy Occurring in the Left Thigh of a 6-Month-Old Female Infant

We report an exceptional case of melanotic neuro-ectodermal tumor of infancy (MNTI) occurring in the soft tissue of the left thigh of a 6-month-old female infant. The tumor consisted mainly of small round cells (neuroblasts) arranged in cords and nests that were separated by broad fibrovascular areas. In addition, there were a few medium-sized tumor cells containing melanin pigment (melano-cytic cells) that in electron microscopy contained melanosomes as well as tonofilaments. Both tumor cell types immunostained for neuron-specific enolase (NSE) and vimentin, and the melanocytic cells reacted additionally with the antikeratin antibody KL1. Within the tumor stroma, neurofilament- and S-100-protein-positive neural cells and vimentin- and desmin-positive myofibroblasts were seen. Although densecore granules were demonstrated ultrastructurally in some neuroblasts, no immunostaining for chromogranin A, Leu-7, serotonin, or regulatory peptides was found. MNTI located in an extremity can be confused with malignant small round and blue cell tumors of childhood. The distinction between MNTI and these tumors is of clinical significance because MNTI, in most cases, is a benign tumor that, in contrast to the latter, can be cured by complete excision. The presence of a biphasic cell population with neuroblasts and melanocytic cells must be considered the main diagnostic feature of MNTI.     

Pigmentary degeneration indwed by N-methyl-N-nitrosourea and the fate of pigment epithelial cells in the rat retina

Pigmentary degeneration of the retina was induced by a single intraperitoneal Injection of 75mgkg of N-methyl-N-nitrosourea (MNU) In female Brown-Norway colored rats at 50 days of age, which were then observed at 24, 48 and 72 h and 7, 21,35 and 150 days after the treatment. MNU-treated rats showed selective destruction of the photoreceptor cells by an apoptotic mechanlsm 24 h after the treatment, and the destruction was completed by day 7. During the photoreceptor cell degeneration, proliferation of Miller cells and infiltratlon of macrophages was prominent 72h and 21 days aRttr the treatment, respectively. Müller cell proliferation and macrophage infiltratbn corresponded to degenerative photo-receptor cell phagocytosis, and prollferating Müller cell processes responded to stabilize the damaged retina. Pigment epithelial cell detachment from the Bruch's membrane was seen 72 h after the treatment, and migration within all layers of the retina was seen at day 7 when photoreceptor Cells were lost. At 21, 35 and 150 days after the treatment, lack of photoreceptor cells and deposition of pigment epithelial cells within the retina but not in contact to vascular endothe-lial cells were characteristic. MNU-induced photoreceptor apoptosis followed by Miiller cell and macrophage reaction then pigment epithellal cells deposition withln the retina partially resembles retinitis pigmentosa in humans.     

The ophthalmic artery and its branches,measurements and clinical importance

Summary Seventy-one Caucasian orbits (36 right, 35 left) were studied by dissection. The diameter of the ophthalmic a. (2 mm from the origin) was 1.54 ± 0.04 mm (male) and 1.31 ± 0.05 mm (female). In individual cases, there were no significant differences in vessel diameter between the right and left sides but, differences in vessel diameter between males and females were more commonly observed in the arteries which leave the orbit (extraorbital group), the individual vessels having a larger diameter in males. The incidence of the ophthalmic a. passing in the orbit medially under the optic n. was 18.6%. The lacrimal a. was observed to arise from the ophthalmic a. in only 82.5% of the cases examined, 15.9% of the cases showed the origin to be at the anastomotic branch of the middle meningealThis article is dedicated to Pr Dr Hoepke on occasion of his 100th birthday     

A patient homozygous for the SCA6 gene with retinitis pigmentosa

The present authors studied a 55-year-old-patient homozygous for the SCA6 gene who experienced frequent attacks of positional vertigo at 37 years of age with subsequent staggering gait and night blindness. Retinitis pigmentosa (RP), as well as cerebellar ataxia and vertical antidirectional nystagmus, were detected. The subject's parents were first cousins, and two of his three male cousins, whose parents were also first cousins, had RP without ataxia or nystagmus. The numbers of CAG repeats in the expanded alleles of the SCA6 gene found by molecular analysis were 21 and 21. The genetic results were negative for SCA1, SCA2, SCA3, SCA7 and dentatorubral pallidoluysian atrophy. The retinal degeneration in this patient is most likely to be secondary to a genetic disorder of autosomal or X-linked recessive inheritance rather than SCA6. Other reported cases of patients homozygous for the SCA6 gene are also reviewed.     

Rhodopsin resynthesis in rats with hereditary retinal dystrophy

Rhodopsin resynthesis was studied in vivo in the retina and optic cup of two strains of rats with hereditary dystrophy: Campbell albino rats and Hunter rats with pigmented eyes. Wistar and MSU rats, respectively, were used as the controls. The rate of reduction of rhodopsin after its decolorization in the retina in the affected animals was shown to be much slower than in healthy animals and to decrease as the disease developed. In the period of marked morphological changes, only 50% of the decolorized pigment was reduced during 2 h of dark adaptation (the time for complete regeneration of rhodopsin in healthy rats). In Campbell and Hunter rats the breakdown and resynthesis of rhodopsin take place not only in the retina, but also in the layer of fragments of outer segments of the photoreceptors, located between cells of the pigmented epithelium and the retina.Laboratory of the Biochemical Basis of Research, I. M. Sechenov Institute of Evolutionary Physiology and Biochemistry, Academy of Sciences of the USSR, Leningrad. Laboratory of Cyctochemistry and Molecular Biology, Institute of Human Morphology, Academy of Medical Sciences of the USSR, Moscow. (Presented by Academician of the Academy of Medical Sciences of the USSR S. E. Severin.) Translated from Byulleten' Éksperimental'noi Biologii i Meditsiny, Vol. 85, No. 2, pp. 167–170, February, 1978.     

The optokinetic nystagmus and ocular pigmentation of hypopigmented mouse mutants

We tested the optokinetic nystagmus (OKN) reflex of various hypopigmented mutant mice and ultrastructurally examined the pigmentation of various ocular structures in these mutants. Using electron microscopy we examined the pigmentation of the choroid and retinal pigment epithelium (RPE) and measured the numerical density, volume density, and distribution of RPE melanosomes of mice with the following phenotypes: (1) wild type, (2) mutants that have abnormal or no OKN in response to horizontally moving, full-field stimulation, and (3) other mutants that have normal OKN but reduced choroidal pigmentation. We also measured the OKN of all these mice in response to horizontally moving stimuli that were restricted to the nasal or to the temporal retina. We found that in the mutants with normal OKN the numerical density of melanosomes in the RPE was within the range found for wild type, while the numerical density was reduced for the mutants with abnormal OKN. For one mutant with normal RPE pigmentation and normal OKN, the choroidal pigmentation was nearly absent. For the genotypes with abnormal OKN the volume density of the RPE melanosomes and percent apical melanosomes were sometimes greater and sometimes less than normal. The OKN patterns of these mice fell into the following categories: (1) wild type; (2) field-restriction dependent OKN with small following movements but no OKN in response to full-field stimulation, normal OKN in response to stimulation of the nasal retina, and OKN of reversed direction in response to stimulation of the temporal retina; (3) oblique with slow oblique following movements and reduced numbers of OKNs with oblique quick phases in response to horizontally moving, full-field stimulation, nearly normal OKN in response to stimulation of the nasal retina, and OKN of reversed direction in response to stimulation of the temporal retina. The horizontal component of the oblique response to full-field stimulation was in the same direction for the two eyes, but the vertical component was in the opposite direction. (4) Slow, small amplitude, with no or very small following movements in response to full-field stimulation, following movements in response to stimulation of the nasal retina and reversed "following" movements in response to stimulation of the temporal retina but few or no quick phases of the OKN for any stimulus condition. These results show that a variety of abnormalities of the OKN occur for hypopigmentation mutants of the mouse.(ABSTRACT TRUNCATED AT 400 WORDS)     

Optic pit syndrome

In a study of 15 eyes with optic pits fluorescein angiography revealed that only pits associated with serous detachment of the macular retina showed fluorescence in the late venous phase, while the pits uncomplicated by detachment remained hypofluorescent throughout the test. This finding strongly supports the hypothesis that the subretinal fluid producing the macular detachment originates from leakage of vessels located in the floor of the pit. In addition a high incidence of anomalies was found to be associated with optic pits. Therefore the author suggests to call the association between optic pit and large optic disc, parapapillary chorioretinal changes, cilioretinal vessels, situs inversus and eventual other vessel abnormalities, the optic pit syndrome.     

Hyperosmolarity response of ocular standing potential as a clinical test for retinal pigment epithelium activity normative data

The standing potential of the eye is decreased by intravenous administration of hypertonic solutions. This hyperosmolarity-induced response has been recorded in normal subjects by the use of electro-oculography (EOG) in the dark. An intravenous administration of Fructmanit^® (1.4 × 10³ mOsmol) (150 500ml, 2.37 9.70ml/kg, 0.08 0.36 ml/kg/min) was used to evoke the hyperosmolarity response. The amplitude of the response was expressed in percentage, V₀ – V_min/V₀ × 100, where V₀ is the base value of the EOG before administration of the hypertonic solution and V_min is a minimum EOG amplitude after administration. The distribution of the amplitude of the hyperosmolarity response was approximated by the normal distribution in normal subjects. The minimum, the maximum, the mean and the standard deviation of the amplitude of the hyperosmolarity response were respectively 34.2%, 52.3%, 42.6% and 4.6% in normal subjects. The normal range of the hyperosmolarity response would be 33.4 51.8% (M ± 2SD). The hyperosmolarity response, which originates mainly in the retinal pigment epithelium, is a useful new quantitative and specific test of the activity of the retinal pigment epithelium in clinical practice.     

Hyperosmolarity response of ocular standing potential as a clinical test for retinal pigment epithelium activity rhegmatogenous retinal detachment

The hyperosmolarity response of the ocular standing potential was recorded in unilateral rhegmatogenous retinal detachment (8 eyes) and in the fellow healthy eye (8 eyes). The hyperosmolarity response was greatly suppressed (M-4 SD: M and SD indicate respectively the mean and the standard deviation in normal subjects) in all affected eyes (p < 0.005), and slightly abnormal in 2 fellow eyes. The L/D ratio was normal in 2 affected eyes and in all fellow eyes. The hyperosmolarity response in the affected eyes was still greatly suppressed 14 months after successful surgical treatment.