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The optic syrinx: cavernous degeneration of the optic nerve demonstrated by iopamidol optic neurography.

Cavernous degeneration of the optic nerve has been described predominantly in association with long-standing glaucoma, although less frequently it has been reported in other, disparate disease processes. The only descriptions to date have been in pathologic specimens in vitro. This is the first report to describe syrinx formation of the optic nerves in vivo in two patients with nonglaucomatous conditions affecting the optic nerves.

Adolescent↗

Presence of LHRH (luteinizing hormone-releasing hormone) fibers in the optic nerve, optic chiasm and optic tract of the adult rat.

In mammals LHRH (luteinizing hormone-releasing hormone) is synthesized and released by a set of neurons that have their embryonic origin in the olfactory placode. We have observed that, besides their classical location, LHRH fibers can also be seen in the optic nerve and optic chiasm. Some LHRH fibers could also be traced in the optic tract. The possible course of these projections, and their functional significance are discussed.

Age Factors↗

The SKILL Card test in optic neuritis: experience of the Optic Neuritis Treatment Trial. Smith-Kettlewell Institute Low Luminance. Optic Neuritis Study Group.

OBJECTIVES: To assess the value of the Smith-Kettlewell Institute Low Luminance (SKILL) Card test, designed to measure vision at reduced contrast and luminance, among patients with previous optic neuritis. MATERIALS AND METHODS: The SKILL Card test was administered to 295 patients participating in the Optic Neuritis Treatment Trial (ONTT) follow-up study, concurrent with measurement of visual acuity, visual field, contrast sensitivity, and color vision. Health-related quality of life (HRQL) was also assessed in a subset of patients using the National Eye Institute Visual Function Questionnaire and an ONTT-developed questionnaire. RESULTS: The SKILL Card difference score (high-contrast acuity score minus low-contrast acuity score) was only weakly associated with the other measures of vision function (rs absolute range, 0.05-0.31) and with the HRQL measures (rs absolute range, 0.02-0.15). In contrast, the light and dark component scores of the SKILL Card test had higher associations with the other vision measures (rs absolute range, 0.27-0.54) and with the HRQL measures (rs absolute range, 0.10-0.40). CONCLUSIONS: The SKILL Card difference score is not a meaningful measure for patients with optic neuritis; however, the test appears to have clinical usefulness as a method to measure high-contrast and low-contrast acuity.

Adolescent↗

GABAergic and non-GABAergic projections of accessory optic nuclei, including the visual tegmental relay zone, to the nucleus of the optic tract and dorsal terminal accessory optic nucleus in rat.

This study examines the non-gamma-amino butyric acid (GABA)ergic (group I neurons) and GABAergic neurons (group II neurons) of the accessory optic system projecting to the nucleus of the optic tract (NOT)/dorsal terminal nucleus (DTN) of the accessory optic system in rat. These nuclei include the dorsal (MTNd) and ventral (MTNv) divisions of the medial terminal nucleus, the lateral terminal nucleus, the interstitial nucleus of the superior fasciculus, the posterior fibers, and the visual tegmental relay zone. GABAergic neurons of these nuclei that do not target the NOT/DTN (group III neurons) have also been observed. The fluorescent retrograde tracer fluoro-gold was injected into the pretectum, targeting the NOT/DTN and the tissue prepared immunocytochemically to reveal neurons containing the neurotransmitter GABA. Three groups of neurons (groups I, II, and III neurons) were examined in terms of their distribution, density, and percentage present. Group I neurons are single-labeled with fluoro-gold and represent non-GABAergic neurons projecting to the NOT/DTN. These neurons are of the highest density in the lateral terminal nucleus (204 neurons/mm2). Their densities are also substantial in the MTNv (120 neurons/mm2), interstitial nucleus of the superior fasciculus, posterior fibers (96 neurons/mm2), and visual tegmental relay zone (93 neurons/mm2). Group II neurons are double-labeled with fluoro-gold and GABA. They form a system of GABAergic neurons projecting to the NOT/DTN, which are exceedingly dense in the MTNd (78 neurons/mm2) but are also dense in both the visual tegmental relay zone (49 neurons/mm2) and MTNv (33 neurons/mm2). Group III neurons are GABAergic neurons that do not target the NOT/DTN but must project to other brain nuclei and/or be interneurons. These are of extremely high concentration in the visual tegmental relay zone (316 neurons/mm2) and are also of substantial densities in the MTNd (77 neurons/mm2), lateral terminal nucleus (72 neurons/mm2), and MTNv (44 neurons/mm2). The MTNd has the highest percentage of GABAergic neurons projecting to the NOT/DTN (72%). GABAergic neurons also form significant percentages of the projections to the NOT/DTN from the visual tegmental relay zone (34%) and MTNv (21%). The percentage of the total GABAergic neurons that project to the NOT/DTN is the highest in the MTNd (50%) and MTNv (42%). The described GABAergic afferents to the NOT/DTN may function to process information concerned with the compensation for retinal slip.

Animals↗

Cavernous hemangioma of optic chiasm, optic nerves and right optic tract. Case report and review of literature.

Diminishing right ocular visual acuity for three weeks in a 30 year old man was confirmed by examination. Bilateral scotomata and bitemporal hemiachromatopsia indicated a chiasmal lesion; reduced visual acuity and Marcus Gunn pupil of the right eye and left relative temporal hemianopia indicated asymmetric involvement. Erythrocytes in the CSF verified a suspected subarachnoid bleed; contrast-enhanced CAT scan demonstrated a suprasellar mass. A cystic, multiloculated, bluish mass distorted the right optic nerve, tract, and chiasm. A hematoma was evacuated and biopsy revealed a cavernous hemangioma of the right optic nerve. Post-operatively, visual acuity has recovered in the right eye but a left homonymous temporal hemianopia has developed.

Adult↗

[Pallor of the optic papilla--an early sign of glaucoma. A clinical controlled study of optic disk pallor and papillar cupping in glaucoma simplex, ocular hypertension and normal eyes with the optic nerve head analyzer].

Double examinations of 99 eyes (34 healthy, 12 with ocular hypertension, 53 with primary open-angle glaucoma) were performed with the Optic Nerve Head Analyzer to evaluate whether an increase in disk pallor or in the cup-disk ratio (CDR) is the earlier sign of glaucoma. In eyes with primary open-angle glaucoma the CDR and the mean optic disk pallor value are significantly higher than in healthy eyes. There is no significant difference in the CDR of patients with ocular hypertension as compared to normals. However, the mean pallor value is significantly higher in eyes with ocular hypertension than in healthy eyes. Therefore, an increase in pallor may precede a significant increase in the CDR or detectable visual field defects.

Glaucoma, Open-Angle↗

Human optic nerve fiber count and optic disc size.

In the optic nerve head, the optic nerve fibers are represented by the neuroretinal rim. The rim area showing a high interindividual variability is positively correlated with the optic disc size. This study was performed to address the question of whether, in addition to having a larger neuroretinal rim, eyes with large optic discs also have a higher count of optic nerve fibers compared to eyes with small optic nerve heads. Histologic semithin sections of 72 optic nerves of 56 cornea donors were histomorphometrically evaluated using a computerized image analyzer. The optic nerve fiber count increased significantly (P = 0.01) with enlarging optic disc size. The nerve fiber count was positively correlated with the retrobulbar optic nerve cross section area. It decreased with advancing age, with a mean annual loss of about 4,000 fibers. The nerve fiber density per disc area decreased with increasing optic disc area. Mean and median of the minimal nerve fiber diameter was larger in older subjects. The results may indicate that the optic nerve fiber count, and the anatomic reserve capacity in progressive optic neuropathies, are higher in eyes with large optic discs than in eyes with small optic nerve heads. The optic nerve fiber population decreased with advancing age. This is important for progression, pseudoprogression, and prognosis of optic neuropathies. Optic nerve fiber crowding is more marked in eyes with small optic discs than in eyes with large optic nerve heads. The age-related loss of predominantly small optic nerve fibers can potentiate the optic nerve atrophy in glaucoma and Alzheimer's disease, with both damaging preferentially large axons.

Adult↗

Pseudophakic accommodation with translation lenses--dual optic vs mono optic.

PURPOSE: To investigate the pseudophakic accommodation effect in dual and mono optic translation accommodative intraocular lenses (AIOL) using linear matrix methods in the paraxial space. METHODS: Dual (anterior optic of power +32 D linked to a compensatory posterior optic of negative power) and mono lens power was determined in the non-accommodated state using linear geometric optics based on the Gullstrand model eye. The position of the AIOL was calculated from a regression formula. Pseudophakic accommodation was assessed with three systems: (1) forward shift of the mono optic lens, (2) anterior translation of the anterior optic in the dual optic lens system with an unchanged position of the posterior minus lens and (3) symmetrical anterior and posterior translation of the anterior and posterior lens. The Gullstrand model eye was modified by changing the axial length (and proportionally changing the phakic anterior chamber depth) to investigate the accommodative effect in myopic and hyperopic eyes. RESULTS: The dual optic lens system (2) yields a nearly constant accommodation amplitude of 2.4-2.5 D mm(-1) movement over the total range of axial lengths. The mono optic lens (1) provides a higher accommodative effect only in extremely short eyes (high refractive power of the lens), whereas for normal eyes (1.4-1.5 D mm(-1) movement) and for long (myopic) eyes the accommodative effect is much less than the dual optic lens. The dual optic lens system under condition (3) yields less accommodation amplitude compared with the dual optic system under condition (2) over the total range of axial length but provides higher accommodation amplitude compared with the mono optic lens system (1) with axial lengths greater than 22.3 mm (lens power 25.5 D). In the accommodated state, with lens translation of 1 mm, the absolute value of the lateral magnification increases with the refractive power of the mono optic lens (1) and decreases in both dual optic lens systems (under conditions 2 and 3). CONCLUSIONS: A mathematical strategy is presented for calculation of the accommodative effect of mono-optic and dual optic AIOL. The dual optic lens yielded a nearly constant accommodation amplitude of about 2.4-2.5 D mm(-1) translation, whereas the mono optic lens yielded an accommodative response of <2 D mm(-1) translation in long myopic or normal eyes. Only in extremely short eyes is the accommodative amplitude of the mono-optic lens higher than the dual optic lens.

Accommodation, Ocular↗

[Optic neuritis--from diagnosis to optic nerve transplantation].

Optic neuritis is a clinical syndrome resulting from inflammation, demyelination, or infection of the optic nerve. Its diagnosis and treatment are complicated. In 1884, Nettleship first reported 28 cases of optic neuritis whose clinical symptoms have been accepted up to the present without any change. On the other hand, the development of diagnostic procedures and steroid therapy have also altered the clinical features of optic neuritis. Among several developed diagnostic procedures, the visually evoked cortical potential (VECP) has become a good tool to prove the impairment of the optic nerve. In 1971, we reported a decrease of threshold intensity required to evoke VECPs in optic neuritis patients whose visual acuity was relatively well preserved. In the same year, Halliday et al. reported that pattern VECP (PVECP) was delayed in 93% of patients with multiple sclerosis (MS) without optic neuritis. Stimulated by this report, a great number of studies appeared to show the usefulness of PVECP in the diagnosis of MS. However, few of these studies gave descriptions of ophthalmic findings. PVECP later become known to be closely related with ophthalmic conditions. In the ophthalmological field, we reported the influence of pupillary size, accommodation power, refractive powers, eccentricity of stimulated retinal area, retinal luminance, contrast, wavelengths, spatial and temporal frequencies, stimulus field etc. On the basis of our results, we developed a television display system in 1975 and applied it clinically. In the present study, we reviewed the medical records of a total of 272 cases of optic neuritis who presented in our clinic between 1978 and 1999. In the diagnostic, therapeutic point of view in relation with the data of other countries, the study was important regarding the racial differences and recent conceptions of optic neuritis. The results showed that there were no racial difference in optic neuritis as had been thought. The development from optic neuritis to multiple sclerosis was not less than in Caucasian patients. Regarding steroid therapy, we found that the most effective method was sub-Tenon injection. For cases which recur and progress to optic atrophy, optic nerve transplantation will be needed. Therefore, we have been studying the reconstruction of the optic nerve in Wister rats. We experimentally damaged the ganglion cells by causing ischemic retina with ligation of the ophthalmic artery and cutting the optic nerve just behind the eyeball. To prevent the apoptosis of ganglion cells, we injected various neurotrophic factors such as BDNF, GDNF, and HSP 27 into the vitreous. For effective injection of DNA, electropolation was applied and the best condition for avoiding apoptosis was chosen. Further, in Mx-c-fos transgenic mice, we found that regeneration of ganglion cells was inhibited. Based on the rescue study of the ganglion cells, optic nerve transplantation was performed using an artificial graft in which cultured Schwann cells from the ischiatic nerve, BDNF, CNTF, insulin, and forscolin were compound and bridged to the superior colliculus. The results showed a regeneration rate of the optic nerve axon of 15%. This rate was much higher than in other reports. Keratoplasty and intraocular lens implantation had a relatively long history of research before achieving clinical success. We believe that optic nerve transplantation will one day be successful in clinical treatment in the same way.

Adult↗

[Recovery of visual field defects in ischemic optic neuropathy and idiopathic optic neuritis].

Fifty-four eyes of 41 patients with optic nerve disease demonstrating acute visual field defects without any traumatic, compressive, or other known etiology were classified into four categories. Those showing poor recovery of visual field defects were ischemic optic neuropathy which was subclassified into either anterior ischemic optic neuropathy (AION) or posterior ischemic optic neuropathy (PION) according to the ophthalmoscopic changes in the optic nerve head. Those showing good recovery of visual field defects were idiopathic optic neuritis which was subclassified into either papillitis or retrobulbar neuritis according to the ophthalmoscopic pathology of the optic disc. Patients with ischemic optic neuropathy were significantly older than those with optic neuritis. All eyes with optic neuritis showed good recovery of vision, whereas those with ischemic optic neuropathy showed varying outcomes of vision. With regard to the pattern of field defect, central or paracentral scotoma was predominant in all but eyes with AION in which altitude defect predominated. Pale swelling of the optic nerve head and angiographic evidence of circulatory disturbance in the optic disc or adjacent choroid were common findings in eyes with AION, whereas such findings were never observed in eyes with papillitis. The amplitude of pattern visual evoked potential was significantly lower in eyes with PION than in those with retrobulbar optic neuritis. Four patients classified as optic neuritis developed into multiple sclerosis in the follow-up study. It was concluded that poor recovery of visual field defect is one of the most convincing evidences for the diagnosis of ischemic optic neuropathy.

Adult↗

Optic nerve fiber count and diameter of the retrobulbar optic nerve in normal and glaucomatous eyes.

BACKGROUND: The caliber of the retrobulbar optic nerve and the count of optic nerve fibers vary considerably in normals. The diameter of the retrobulbar optic nerve also decreases with optic nerve atrophy. This study aimed to determine the relationship between the caliber of the optic nerve and the optic nerve fiber count. METHODS: We counted the optic nerve fibers and measured the diameter of histological cross sections of the optic nerve for 56 normal subjects and 23 patients with absolute glaucoma. RESULTS: The optic nerve fiber count increased significantly (P < 0.0001) by 777,000 fibers for every millimeter increase in retrobulbar optic nerve diameter, starting at a baseline diameter of 1.89 mm. CONCLUSION: Using this linear regression equation, the optic nerve fiber count can be estimated in routine histology by measuring the optic nerve diameter. Taking into account a fixation-induced tissue shrinkage, this method may also give some indication of the optic nerve fiber count intravitally, when, for eyes with opaque optic media, the diameter of the retrobulbar optic nerve has been measured by imaging techniques. Greater retrobulbar optic nerve caliber may indicate greater structural reserve capacity.

Adult↗

A serial MRI study following optic nerve mean area in acute optic neuritis.

This study assessed optic nerve mean area on serial MRI in a cohort of patients with a first episode of acute unilateral optic neuritis to assess the effects of a single acute inflammatory demyelinating lesion. Twenty-nine patients with a median delay from onset of visual symptoms of 13 days (range 7-24 days) were recruited. After a clinical examination and visual evoked potential (VEP) measurement, each patient had their optic nerves imaged with a coronal fat-saturated short echo fast fluid-attenuated inversion recovery sequence. Twenty-one patients had serial examinations after 2, 4, 8, 12, 26 and 52 weeks. In addition, 32 control subjects had their optic nerves imaged up to three times. The mean cross-sectional area of the intra-orbital portion of each optic nerve was calculated by a blinded observer using a computer-assisted contouring technique. At baseline, the mean area of diseased optic nerves was 16.1 mm2 compared with 13.4 mm2 for healthy contralateral optic nerves (20.1% higher, P < 0.0001) and 13.6 mm2 for controls (18.4% higher, P = 0.0003). The diseased optic nerve mean area declined over time, from initial swelling to later atrophy. The mean decline at 52 weeks was -0.0018 mm2/day (95% confidence interval -0.0038 to -0.00051). At 52 weeks, the mean area of diseased optic nerves was 11.3 mm2 compared with 12.8 mm2 for healthy contralateral optic nerves (11.7% lower, P = 0.032) and 13.1 mm2 for controls (13.7% lower, P = 0.008). The 52 week diseased optic nerve mean area was not significantly affected by the baseline mean area. There was an association between baseline optic nerve mean area and logMAR visual acuity (rS = 0.46, P = 0.012) and visual field mean deviation (rS = -0.55, P = 0.002), but there was no evidence of an association between 1 year mean area and visual outcome. There was no evidence of association between baseline, rates of decline or 1 year diseased optic nerve mean areas and any of the baseline, 1 year or time-averaged VEP variables. The present study shows a consistent pattern of changes associated with individual inflammatory demyelinating lesions in the optic nerve. Acutely, there was swelling, consistent with the presence of acute inflammation, which was related to visual impairment. Over the longer term, there was loss of tissue. The lack of association between 1 year optic nerve mean area and visual outcome may reflect a mild loss of tissue, redundancy or remodelling of function.

Acute Disease↗

Normal and regenerating optic fibers in goldfish tectum: HRP-EM evidence for rapid synaptogenesis and optic fiber-fiber affinity.

The distribution of normal and regenerating retinal fibers and synapses was studied on tectum in goldfish by light (LM) and electron microscopy (EM). Since labeling of the early regenerating fibers was previously reported to be difficult, a new 'cold-fill' HRP labeling protocol was developed, which labeled regenerating optic fibers and terminals on tectum as early as 14 days after nerve crush when they first arrive on tectum. In order to characterize the laminar distribution of optic afferents in normal fish and in fish regenerating for 14-240 days, EM photomontages of areas 14 microns wide by 160 microns deep through the HRP-labeled primary optic innervation layer (S-SO-SFGS) were constructed. The time points in regeneration that were examined spanned the period in which others have shown that an initially diffuse retinotopic map becomes spatially restricted. At the LM level regenerating optic fibers were restricted to the optic lamina. They reinnervated tectum in an anterior to posterior sequence as previously seen with autoradiography. In addition, at 14 days, some "pioneer" optic fascicles were found to have already grown to posterior tectum where they gave rise to branches with boutonlike terminations and growth-cone-like processes. Form the ultrastructural analysis it was clear that optic fibers and terminals observed strict laminar boundaries as they partitioned themselves in the optic laminae (S, SO and SFGS) in both normal and regenerating fish. The behavior of optic fibers was lamina specific with respect to synapse formation and the orientation of fiber outgrowth. As early as 14 days regeneration, optic fibers made synapses onto the four types of postsynaptic profiles observed in normal fish. Numerous optic terminals were labeled at 14 days, and there appeared to be no waiting period between fiber ingrowth to the SO and synapse formation in the S and SFGS. At 14-60 days, atypical synaptic contacts which appear to be nascent synapses were made by labeled optic fibers in fascicles and by growth-cone-like processes. By 21-30 days, the density of optic terminals was high and there were many more fasciculated optic fibers in the SFGS than normal as late as 350 days. These findings suggest that optic fiber lamination is highly constrained by tectal cues, that fibers rapidly regenerate many synaptic terminals before retinotopic map refinement is complete, and that fibers have a strong affinity for each other.

Animals↗

The distribution of axons according to diameter in the optic nerve and optic tract of the rat.

The distribution of axons according to diameter was examined in the optic nerve and optic tract of adult hooded rats. Observations were made on semithin sections, and measurements of axonal diameters were made on electron micrographs taken from various locations across thin sections through the optic nerve and tract. The distribution of axons by size differs markedly in the optic nerve and tract. Coarse (greater than 2 microns) and fine (less than or equal to 2 microns) axons are distributed throughout all regions of the optic nerve. In the optic tract, in contrast, coarse axons are especially dense dorsally, at the deep border of the tract, while they are absent ventrally, subjacent to the pial surface. No regions of the optic nerve contain densities of coarse axons as high as the deep nor as low as the superficial extremes of the optic tract. Nevertheless, even at the deep (dorsal) border of the optic tract, the coarse axons make up only a small minority (roughly 15%) of the total number of axons in that region. The axons 2 microns or smaller may be divisible into two overlapping, fine and intermediate, diameter classes, that are partially segregated within the optic tract, but not in the optic nerve: the distributions of axon diameters smaller than 2 microns are skewed to distinctly smaller diameters at the dorsal and ventral extremes of the optic tract, while in between, at mid-positions along the deep-to-superficial axis of the optic tract, the axon size distributions contain many more axons greater than 1 micron in diameter. These different axon diameter groups may arise from the morphologically distinct retinal ganglion cell types, and may underlie the components of the trimodal compound axon potential seen in the rat's primary optic pathway. Their partial segregation within the tract anticipates the partial segregation of their terminal arborizations within the laminae of the dorsal lateral geniculate nucleus. The rearrangement of axons into a partial segregation by size within the optic tract may indicate a chronology of axonal arrival during early development, proximity to the pial surface being an index of recency of arrival. As axonal outgrowth and neurogenesis appear to be directly related within the retinal ganglion cell population in mammals, the relative birthdates of the retinal ganglion cell types giving rise to the axon diameter classes in the rat may be inferred from the present results.

Animals↗

Vasculature and morphometry of the optic canal and intracanalicular optic nerve.

OBJECTIVES: To study the bony structure of the optic canal and the vasculature of the intracanalicular optic nerve in human cadavers. MATERIALS AND METHODS: Gross and microscopic examinations were performed in 25 optic canals from 13 cadavers to study the pattern of vascular supply of the intracanalicular optic nerve. Neoprene latex was injected through the most proximal part of the ophthalmic artery in seven optic canals. The intracanalicular branches from the ophthalmic artery were carefully identified and quantified. Quantitative measurements of the canal length, canal thickness, canal transverse area, optic nerve transverse area, and subdural space were done for the other 18 canals by means of semiautomated morphometric analysis system. Each canal was divided into anterior, middle, and posterior parts for better visualization and measurement. RESULTS: The ophthalmic artery gives off three branches that supply the intracanalicular optic nerve: medial collateral branch, lateral collateral branch, and ventral branch. Each branch pierces the dura and then supplies the nerve through the pia mater. The middle medial wall was the thinnest bony part of the canal (0.31 +/- 0.06 mm). The optic canal, optic nerve, and subdural space transverse area varied at different transection levels. The narrowest space was in the middle part of the optic canal. The mean subdural cross-sectional space was only 1.84 mm2. This, multiplied by the average canal length (11.79 mm), can be considered the potential space for hemorrhage, optic nerve edema, or hematoma. CONCLUSIONS: The vasculature within the bony canal is extremely delicate. Due to the limitation of this space, even a tiny amount of blood or swelling of the nerve (21.69 mm3) may cause optic nerve compression. It appears that these vessels could easily be disrupted in closed head injury by a shearing or concussive force, leading to ischemic infarction of the optic nerve. Since the narrowest portion of the canal is in the middle portion, it is the middle part of the optic canal that is most critical in doing an optic canal decompression.

Anthropometry↗