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At least 721 records · Page 40Linked to original sources
PubMed1981

[Electroencephalogram in congenital anophthalmia (author's transl)].

Dysgenesis of the eye produces occipital needle sharp spikes. Neither the age and maturational state of the eye nor the brain create the pathophysiologic conditions which give rise to needle sharp spikes. It seems rather that deafferentation of the visual cortex gives rise to an increase in its irritability which make it fire off in this special manner. Cannon and Rosenblueth presented evidence that, as a general rule, deafferented systems tend to become hyperexcitable.

Adolescent↗
PubMed1981

[Neurocristopathies of the human nasofrontal bud. Ethmoidal syndromes (hypo- and hyper-septoethmoidism) (author's transl)].

Knowledge of human brain development biology has increased rapidly over the last decade due to fundamental acquisitions in the fields of organogenesis, fetal development, and post-natal growth. The role of the neural crests during cephalic organogenesis, recently studied by Mme Nicole Le Douarin, has given new biological dimensions to the head : it is an organic complex, globally neural in character, and of encephalofacial territorialization. The cervicocephalic region derives this original segmentation from the migratory property of this contingent of the neural crests. In this perspective, the nasofrontal bud is therefore an embryonic cephalic segment, in which appears the pro-encephalon, the eyes, the olfactory bulb, and the median facial zones (bone, cartilage, teeth). Any morphogenetic anomaly in this original craniofacial segment creates associated malformation of the brain, the base of the skull, and the face (eyes and olfactory bulbs) : the neurocristopathies. In this respect, and from the semiological point of view, the face is the "predicate" of the brain. A new classification of these malformations is proposed, based on the clinical study of the ethmoid, a cartilaginous median organ which is accessible clinically. Epigenesis lasts for 18 months in this organ, which forms a part of the face and the neurocranium, and which is known to play a "motor" morphogenetic role on the osteomembranous face : the ethmoidal syndromes (hypo- and hyper-septoethmoidism). Future reports will discuss the neurocristopathies of the branchial arches.

Abnormalities, Multiple↗
PubMed1982

Differentiation of the optic cups from an anophthalmic murine strain, in culture and in intrafoetal grafts.

The optic anlage of the anophthalmic murine strain ZRDCT/an has been studied. In this strain, 70% of the adults are anophthalmic, 30% are one-eyed. The eye primordium appears and starts to differentiate at day 9 of gestation, then it degenerates. At 13 days of gestation, it has practically disappeared. When isolated and cultured in vitro, the 10-day anophthalmic anlage develops in the same way as a control optic-cup culture from a Swiss or C57B16 mouse. When grafted in the back of a 15--17 day Swiss foetus, this 10-day anophthalmic anlage also develops: retina and lens differentiate, the structures which grow are very similar to those obtained when C57B16 optic cups are grafted in the same conditions. It appears from these results that the eye regression of the mutant in situ is not a process intrinsic to the anlage. If withdrawn from its local environmental influences, the eye primordium is able to express its aptitude for differentiation.

Animals↗
PubMed1980

Anophthalmia in an infant of parents using LSD.

We have described a unilateral anophthalmia in an infant with normal chromosome studies born to parents who took LSD before and during pregnancy. In view of the lack of any other fetal abnormalities in this child, known to be associated with LSD, we report this case to suggest that anophthalmia may be a new drug-related defect. A complete drug history of the parents should be taken in cases of congenital abnormalities to accumulates further data, which will point conclusively to the etiology of anophthalmia and possibly other congenital defects.

Abnormalities, Drug-Induced↗
PubMed1980

Mental retardation and unilateral anophthalmia in hemifacial microsomia.

A 3-year-old boy with left anophthalmia and hemifacial microsomia was found to be not severely retarded. Previous reports have emphasized concomitant severe mental retardation in patients with this malformation complex. Review of the literature and the present case indicate that mental retardation is a variable feature of this condition.

Anophthalmos↗
PubMed1994

Effect of pre- and postnatal retinal deprivation on the striate-peristriate cortical connections in the rat.

The tangential distribution of the striate-peristriate cortical connections in normal, postnatally eye enucleated and congenitally anophthalmic rats, was studied after a single injection of wheat germ agglutinin conjugated with horseradish peroxidase into the striate cortex. The typical normal pattern of separate fields in the peristriate cortex is altered in eye enucleated animals, in such a way that their areal distribution in the cerebral cortex is increased and each field tends to fuse with the adjacent one. This process is more marked in anophthalmic animals, a finding that is in agreement with the notion that ganglion cells exert their influence before the visual pathway is functional.

Animals↗
PubMed1995

Current techniques of enucleation: a survey of 5,439 intraorbital implants and a review of the literature.

Enucleation is a commonly performed procedure. A multitude of intraorbital implants are available for use following enucleation. Each has advantages and disadvantages. This survey report represents the most comprehensive evaluation yet of the current trends in the rehabilitation of the anophthalmic socket. The membership of the American Society of Ophthalmic Plastic and Reconstructive Surgeons (ASOPRS) was surveyed. Information was requested regarding preferred intraorbital implants, complications associated with each implant, and some details of current surgical techniques. Hydroxyapatite (HA) was used in 56% of primary enucleations by this group as compared to 1% in 1989. In comparison to other leading implants, HA was found to have equally low or lower complication rates when poor motility, infection, extrusion, migration, superior sulcus deformity, enophthalmos, lower lid malposition, and contracted fornices were assessed. A detailed discussion of these various complications is presented. The majority (59%) of ophthalmic plastic surgeons use donor sclera when placing an intraorbital implant. HA was clearly the implant material most used by ophthalmic plastic surgeons in 1992, and may be the material of choice for enucleation. To date it has achieved better postoperative results and a favorable side effect profile. Because it has been available for only 5 years, more time is necessary to determine its long-term success rate. Despite theoretical concerns of the transmission of infectious agents with the use of donor sclera, the majority of surgeons use this material when placing an intraorbital implant.

Anophthalmos↗
PubMed1995

Distribution and density of monoamine receptors in the primate visual cortex devoid of retinal input from early embryonic stages.

Developmental mechanisms that regulate the areal and laminar distribution of various macromolecules, including neurotransmitter receptors in the cerebral cortex, are not known. In the present study, we examined the development of monoaminergic receptors in the rhesus monkey striate and peristriate visual cortex in the absence of input from the retina. Binocular enucleation was performed between embryonic days E60 and E81, prior to the ingrowth of geniculocortical fibers into the cortical plate and before genesis of the granular and supragranular layers of the visual cortex. The animals were delivered at term (E165) and sacrificed at 2 or 12 months of age, and their brains frozen and the occipital lobes cut at 20 microns in the coronal plane. Cortical binding of 3H-clonidine, 125I-pindolol, 3H-5-HT, 3H-ketanserin, 3H-spiperone, 3H-SCH23390, and 3H-prazosin that label various monoamine receptors were autoradiographically visualized and quantified using a computer imaging system. All radioligands displayed specific laminar patterns in the striate and prestriate areas in both groups of animals. The areal and laminar distribution in the anophthalmic monkeys was similar to that in the controls. Significantly, in all enucleated animals, just as in the controls, a particularly high density of 3H-clonidine and 3H-prazosin was observed in the sublayers of layer IV involved in color vision. The present results show that the monoamine receptors in primate visual cortex can establish and maintain distinct laminar and areal patterns in the absence of activity or molecular cues originated from the retina, and provide new insight into the cortical consequences of secondary congenital anophthalmia.

Animals↗
PubMed1993

The genes involved in the morphogenesis of the eye.

The two main ways to identify the genes involved in the process of morphogenesis are: 1) to analyze genetic causes in patients or animal models with developmental anomalies and 2) to elucidate patterns of the expression of genes by in situ hybridization during the normal development. In this report, these two methods were adopted to identify the genes involved in the morphogenesis of the eye. A unique mutation of the paired box (Pax)-6 gene (a kind of homeobox gene) was found in a new rat strain "rSey" which showed no induction of both lens and nasal placodes in the homozygote. One base "G" insertion in an exon of the genomic DNA gave rise to a new sequence, "GT", which served as an abnormal 5' splice site to generate an internal deletion in the messenger RNA. The homozygote was also known to have impaired migration of neural crest cells from the anterior midbrain, indicating that the Pax-6 gene would play a role in conducting migration of these neural crest cells. Neural crest-derived mesenchymal cells located around the lens vesicle in the optic cup of mouse embryos were positive for the expression of retinoic acid receptor genes, showing that retinoic acids played a role in the formation of such eye structures composed of neural crest-derived cells as the primary vitreous, corneal, iris, and ciliary stroma. Transcripts of the fibroblast growth factor receptor type 1 were found mainly in neuroepithelium of the optic cup of chick embryos, whereas those of the type 2 receptor were detected in neural crest-derived mesenchymal cells surrounding the optic cup. In contrast, the type 3 receptor was expressed mainly in the lens vesicles, suggesting that altogether 3 types of the fibroblast growth factor receptor would be involved in signaling among different structures as the optic cup, lens vesicle, and neural crest-derived mesenchyme. Receptors for activin, a member of the transforming growth factor beta superfamily, were expressed in the neuroepithelium of the optic cup and in the lens vesicle. In the light of these molecular biological findings, the roles of neural crest-derived cells and epithelial-mesenchymal interactions as well as the concept of positional information in the morphogenesis of the eye were discussed.

Activin Receptors↗
PubMed1994

Fraser (Cryptophthalmos-syndactyly) syndrome: a case with bilateral anophthalmia but presence of normal eyelids.

A new case of Fraser syndrome with abnormalities of the genital, urinary and skeletal system is reported. The female newborn died shortly after birth. In contrast to cases of Fraser syndrome reported earlier and to widely accepted theories of eye development the present case presented with normal bilateral eyelids although no tissue of ophthalmic origin was found in the orbital cavities on histological examination.

Anophthalmos↗