1/33. retinitis pigmentosa, nanophthalmos, and optic disc drusen: a case report.OBJECTIVE: Although the associations of retinitis pigmentosa (RP) with nanophthalmos/microphthalmos and RP with optic disc drusen have previously been recognized, the concurrence of all three features, as far as the authors are aware, has not previously been reported. DESIGN: Case report. RESULTS: The authors report a sporadic case of nanophthalmos, RP, and optic nerve drusen with the additional complication of chronic angle closure glaucoma. CONCLUSIONS: Visual loss may be secondary to the complications of nanophthalmos, RP, or optic nerve drusen. Chronic angle closure may be caused by choroidal effusion with serous retinal detachment, which may, in turn, cause a pseudo-RP picture. It is therefore important to recognize the possible association of true RP with nanophthalmos as a cause for visual deterioration.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/33. Atypical retinitis pigmentosa masquerading as a nerve fiber bundle lesion."Sector" or "asymmetric" retinitis pigmentosa was mistaken as bilateral nerve fiber bundle defects in a 56-year-old man for six years, leading to an unnecessary work-up for anterior visual pathway disease and to an incorrect diagnosis of low-tension glaucoma. confusion arises because this variant may present with bilateral, superior, half-ring visual field defects that intersect the blindspots. These defects may be misplotted as typical arcuate Bjerrum's scotomas. The ophthalmoscopic changes that correspond to these visual field defects may be so subtle as to be overlooked. fluorescein angiography helps bring out the retinal lesions, and moderately subnormal electroretinographic and dark adaptation studies are definitive.- - - - - - - - - - ranking = 2.5keywords = nerve (Clic here for more details about this article) |
3/33. Histopathologic features of the temporal bone in usher syndrome type I.Temporal bones of 2 patients with Usher syndrome type I were examined using light microscopy. In both patients, findings from histopathologic examination of the cochlea were characterized by degeneration of the organ of corti, which was most marked in the basal turn, atrophy of the stria vascularis, and a decrease in the number of spiral ganglion cells. The cochlear nerve appeared to be diminished. The sensory epithelium of the saccular and utricular maculae of patient 1 was normal for age. The left temporal bone of patient 2, classified as Usher syndrome genetic subtype USH1D or USH1F, demonstrated the typical signs of severe cochleosaccular degeneration. Present cases and cases from the literature were reviewed in search of an explanation for the above-described differences in histologic findings.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
4/33. The microsystems based visual prosthesis for optic nerve stimulation.The microsystems based visual prosthesis (MiViP) visual prosthesis generates visual perceptions well below safety and stimulator saturation limits. These perceptions, called phosphenes, are of reasonably small size and are broadly distributed in the visual field. They can thus be used to convey useful visual information. Psychophysical evaluations are being performed in order to assess the implantee's benefits in the use of the MiViP optic nerve visual prosthesis. In a pattern-recognition task, the performance improved regularly with practice with an increasing score and a decreasing delay to recognition. These observations open the way toward an evaluation of general mobility improvement with the portable system. In conclusion, the results obtained so far still support the potential usefulness of the optic nerve visual prosthesis. A low-resolution artificial vision can be expected from the prosthesis after extensive training.- - - - - - - - - - ranking = 3keywords = nerve (Clic here for more details about this article) |
5/33. A new familial syndrome characterized by pigmentary retinopathy, hypogonadism, mental retardation, nerve deafness and glucose intolerance.Three siblings with retinitis pigmentosa, deafness and mental retardation were studied. Physical abnormalities included nystagmus, acanthosis nigricans and multiple keloids. The two male siblings had gynecomastia, small testes and mild subvirilization whereas the only indication of hypogonadism in the female sibling was oligomenorrhea. testosterone levels in the males, which were in the low to low normal range, were increased by the administration of large doses of chorionic gonadotropin. The two affected males had elevated plasma luteinizing hormone (LH) and follicle-stimulating hormone (FSH) levels which were decreased by the administration of testosterone and increased by the administration of clomiphene. One sibling had mild obesity and diabetes mellitus, one had moderate obesity, normal glucose tolerance and hyperinsulinism and the third had abnormal glucose tolerance and hyperinsulinism. This familial syndrome is distinct from either the Laurence-moon, Bardet-Biedl or Alstrom disorders and provides further evidence of genetic heterogeneity in this group of autosomal recessive traits.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
6/33. Position, size and luminosity of phosphenes generated by direct optic nerve stimulation.Pulses of low intensity current, delivered through a cuff electrode chronically implanted around the optic nerve of a blind retinitis pigmentosa patient generate visual sensations. These phosphenes are obtained at lower thresholds for a train of stimuli than for single pulses, which suggests the existence of a spatial and temporal integrating mechanism. The perceptions are much smaller than those predicted from model simulations. A set of equations are derived which show the effect of pulse current, duration, number and frequency on the position, size and, to some extent, luminosity of the resulting phosphenes.- - - - - - - - - - ranking = 2.5keywords = nerve (Clic here for more details about this article) |
7/33. Recurrent superior oblique myokymia in a patient with retinitis pigmentosa.Superior oblique myokymia is an infrequently encountered condition, presenting with episodes of oscillopsia and/or vertical or oblique nystagmus, accompanied by a fine, monocular, cyclorotational nystagmus. Recent research suggests it is caused by vascular compression of the trunk of the trochlear nerve. The clinical features of a patient reporting three episodes of superior oblique myokymia, each following childbirth, are described. She had previously been diagnosed with retinitis pigmentosa. The possible aetiologies of superior oblique myokymia are described and appropriate assessment and possible referral for further testing detailed.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
8/33. Peripheral sensory-motor polyneuropathy, pigmentary retinopathy, and fatal cardiomyopathy in long-chain 3-hydroxy-acyl-coa dehydrogenase deficiency.An 11-month-old girl presented acute episodes of hypoglycaemia and hepatic encephalopathy reminiscent of reye syndrome and 3-hydroxydicarboxylic aciduria. The patient showed peculiar clinical manifestations of severe sensory-motor neuropathy, pigmentary retinopathy, and cardiomyopathy. She died of cardiac failure. Pathological studies of peripheral nerve showed signs of axonal neuropathy and demyelination. Enzymatic studies in cultured fibroblasts showed a deficiency of mitochondrial long-chain 3-hydroxyacyl-CoA-dehydrogenase. Peripheral nerve involvement and retinal pigmentary degeneration have as yet not been described in patients with proven defects of mitochondrial beta-oxidation.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
9/33. A new case of oculoectodermal syndrome.An 11-month-old infant girl presented with right-sided features of aplasia cutis congenita of the scalp, unilateral epibulbar dermoids, eccentric pupil, coloboma of the right upper eyelid, and depigmentation of the fundus surrounding the right optic nerve. These findings were similar to the oculoectodermal syndrome reported by other clinicians and researchers.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
10/33. Intraorbital implantation of a stimulating electrode for an optic nerve visual prosthesis. Case report.research into visual prosthetics is expected to revolutionize the treatment of blind patients with incurable outer retinal degenerative disease. Substantial evidence shows that useful visual sensations can be produced by controlled electrical stimulation of the optic nerve. To make the optic nerve visual prosthesis more acceptable, implantation techniques safer and less invasive than those previously used have been developed. A medial transconjunctival approach is now used to implant a stimulating electrode around the intraorbital section of the optic nerve. This new technique allows sufficient exposure of the nerve after detaching only one rectus muscle and performing a lateral canthotomy. Previously, an electrode was implanted in the intracranial part of the optic nerve, which required more invasive surgery. The new technique was first developed in cadavers and in patients undergoing eye enucleations. Finally, a 68-year-old blind man suffering from retinitis pigmentosa underwent long-term implantation. In this case report the authors describe the technique and outline some of the challenges involved.- - - - - - - - - - ranking = 4.5keywords = nerve (Clic here for more details about this article) |
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