Studies on the profile of immunostimulant activities of modified iridoid glycosides

PURPOSE: To determine the prevalence of and risk factors for superior segmental optic nerve hypoplasia in offspring of mothers with type I diabetes mellitus. METHODS: Thirty-four subjects aged between 4 and 37 years, the children of 23 mothers who had type I diabetes mellitus at the time of pregnancy, aged between 4 and 37 years, were recruited from one institution and prospectively examined in search of an optic disk-related anomaly. RESULTS: Of the 34 subjects, three females (8.8%) showed classic ophthalmoscopic and perimetric features of superior segmental optic nerve hypoplasia, bilateral in two patients and unilateral in one. Pregnancies leading to affected children showed a tendency to be shorter, birth weight to be lower, and control of maternal diabetes mellitus to be poorer compared with pregnancies resulting in unaffected children. No variable unique to the affected subjects as opposed to the unaffected majority could be identified. CONCLUSIONS: We found a superior segmental optic nerve hypoplasia, described as a "topless disk," in three of 34 subjects (8.8%) at risk for this condition. Topless disk thus seems to be more common than was previously thought, possibly having been missed because of its subtle signs and only mild impairment of visual performance in affected individuals. Female sex, short gestation time, low birth weight, and poor maternal diabetes control may represent additional risk factors for the development of a topless disk. Its pathogenesis remains obscure, but the responsible pathogenic event may occur in the perinatal period.     

The effect of immunotoxins directed against CD80 and CD86 on primary T-cell alloresponses

A 13-year-old boy presented with acute loss of vision in his right eye of 2 weeks' duration. He had a high fever and was ill for several days, then improved but suffered recurrent episodes of sweating and a high fever. Ophthalmoscopy of the right eye showed optic disk edema, mild vitreous cells, and minimal exudates in the macula. Bartonella henselae titers were positive. A diagnosis of optic disk edema with a macular star secondary to cat-scratch disease was made. The patient was treated with doxycycline and made a dramatic improvement to visual acuity of 20/30 with a minimal residual relative central scotoma. The optic disk edema and macular star resolved, and the patient was left with mild optic atrophy in the right eye.     

Evaluation of coexisting optic nerve head drusen and glaucoma with optical coherence tomography

OBJECTIVE: Optic nerve head drusen often make evaluation of the nerve head difficult to interpret. In addition, visual field defects are known to occur in patients with optic disk drusen, resembling glaucomatous damage. The authors report two cases of coincident optic nerve head drusen and glaucoma, in which the use of optical coherence tomography (OCT) in evaluating the nerve fiber layer was beneficial. PARTICIPANTS: Two patients with both optic nerve head drusen and glaucoma, one with primary open angle glaucoma, the other with pseudoexfoliation glaucoma were evaluated. Both patients had asymmetric optic disk drusen, with clinically visible drusen only in one eye. INTERVENTION: Ophthalmologic examination, color and red-free photography, automated Humphrey visual field testing and OCT were performed. RESULTS: Nerve fiber layer loss as measured by OCT was found to be greater than expected by the appearance of the optic nerve head and red-free photography, with visual fields consistent with findings in case 1. In case 2, visual fields were full, despite nerve fiber layer thinning seen by OCT and red-free photography. CONCLUSIONS: There can be significant nerve fiber layer thinning in patients with both glaucoma and optic disk drusen, despite the appearance of the optic nerve head in these patients. The cup margin may be obscured by the drusen, giving rise to a falsely full-appearing disk. In such cases, OCT may provide a useful means to quantitatively measure the nerve fiber layer thickness and to aid in the management of these patients by detecting nerve fiber layer thinning earlier than would otherwise be possible.     

Bilateral disc edema in retinitis pigmentosa

Retinitis pigmentosa (RP), one of the most common forms of hereditary retinal degeneration, is characterized by night blindness and constricted visual fields. In addition to bone spicule pigmentation, other ocular findings may include posterior subcapsular cataracts, cystoid macular edema, and hyaline bodies or drusen of the optic nerve. Rarely, optic nerve head (ONH) edema has been reported to be associated with RP. A 44-year-old white male with RP and neurosensory hearing loss (Usher's syndrome type II) presented to our clinic for routine examination. A dilated fundus examination revealed bone spicule pigmentation, vessel attenuation, several flame hemorrhages on or adjacent to the nerves, and ONH edema in the right eye. B-scan ultrasonography revealed drusen of the right ONH but not of the left. Late stage fluorescein angiography showed hyperfluorescence and dye leakage from both optic discs which was more pronounced in the right eye than the left. Computed tomography (CT) of the head and orbits and cerebrospinal fluid (CSF) examination by lumbar puncture were normal. The differential diagnosis of bilateral ONH edema in this case included ONH drusen or papilledema secondary to increased intracranial pressure. This patient was found to have RP with asymmetric, bilateral ONH edema of unknown cause. One theory regarding the cause of the ONH edema is disc vessel leakage secondary to an inflammatory reaction caused by rapid photoreceptor and retinal pigment epithelium (RPE) degeneration.     

Current status of role of oxygen in retrolental fibroplasia

Three patients with long-standing diabetes manifested reduced visual acuity and notable bilateral symmetrical cystoid maculopathy, with no other sign of meaningful background diabetic retinopathy, eg, minimal to no microaneurysms, hemorrhages, or exudates. Results of fluorescein angiography showed that the entire retinal capillary bed in the macula was dilated and leaked fluorescein diffusely and profusely, but also showed that there was excellent capillary perfusion. Argonlaser photocoagulation was applied to one eye of each patient in an attempt to resolve the cystoid maculopathy. The laser treatment effectively resolved the edema in the specific areas of treatment, but the foveal and perifoveal areas, which were not treated, showed no areas of resolution of the cystoid edema in two cases and no improvement (and no decrease) in visual acuity in all three cases.     

Unilateral testing of utricular function

PURPOSE: To examine acquired pit of the optic nerve as a risk factor for progression of glaucoma. METHODS: In a retrospective longitudinal study, 25 open-angle glaucoma patients with acquired pit of the optic nerve were compared with a group of 24 open-angle glaucoma patients without acquired pit of the optic nerve. The patients were matched for age, mean intraocular pressure, baseline ratio of neuroretinal rim area to disk area, visual field damage, and duration of follow-up. Serial optic disk photographs and visual fields of both groups were evaluated by three independent observers for glaucomatous progression. RESULTS: Of 46 acquired pits of the optic nerve in 37 eyes of 25 patients, 36 pits were located inferiorly (76%) and 11 superiorly (24%; P < .001). Progression of optic disk damage occurred in 16 patients (64%) in the group with acquired pit and in three patients (12.5%) in the group without acquired pit (P < .001). Progression of visual field loss occurred in 14 patients (56%) in the group with acquired pit and in six (25%) in the group without pit (P=.04). Bilateral acquired pit of the optic nerve was present in 12 patients (48%). Disk hemorrhages were observed more frequently in the group with acquired pit (10 eyes, 40%) compared with the group without pit (two eyes, 8%; P=.02). CONCLUSION: Among patients with glaucoma, patients with acquired pit of the optic nerve represent a subgroup who are at increased risk for progressive optic disk damage and visual field loss.     

Clinical and ultrastructural ocular histopathologic studies of adult-onset metachromatic leukodystrophy

A twin brother and sister with adult-onset metachromatic leukodystrophy developed progressive central acuity loss and optic disk pallor. Both had normal electroretinograms and fluorescein angiography. Postmortem examination of the sister's eyes by histochemistry and electron microscopy revealed ganglion cell loss and optic atrophy. Cerebroside sulfate had accumulated in optic nerve glial cells. Optic atrophy was more advanced than in previously reported cases of infantile-onset metachromatic leukodystrophy. The pathologic process seemed to be retrograde optic nerve degeneration due to abnormal myelin metabolism.     

Change in the land vertebrate community under new conditions in nature]

PURPOSE: To report bilateral congenital optic nerve head pits in monozygotic siblings. METHOD: Case reports. RESULTS: Pits were found in abnormally large optic disks in both eyes of two otherwise healthy female monozygotic siblings aged 15 years. Pit size increased and visual acuity decreased with increased optic disk area. In one eye, nonrhegmatogenous retinal detachment developed that eventually necessitated pars plana vitrectomy. The siblings' parents were unremarkable. CONCLUSIONS: Congenital optic nerve head pits can occur bilaterally in otherwise healthy monozygotic siblings with ophthalmologically unremarkable parents. Associated nonrhegmatogenous retinal detachment may be treated by pars plana vitrectomy. Pit size is positively correlated with disk area.     

High-resolution magnetic resonance imaging of the intraorbital optic nerve and subarachnoid space in patients with papilledema and optic atrophy

OBJECTIVE: To evaluate the orbital portion of the optic nerve and the subarachnoid space using fast spin-echo magnetic resonance imaging in normal subjects and in patients with papilledema or optic atrophy. DESIGN: Measurements of the optic nerve complex on coronal images were made using high-resolution magnetic resonance imaging with fast spin-echo sequences. PATIENTS: Twenty-one patients, including 5 patients with papilledema due to congenital hydrocephalus, intracranial tumors, or meningitis, as well as 16 patients with optic atrophy, were studied. Sixteen healthy volunteers served as controls. MAIN OUTCOME MEASURES: The longitudinal diameter of the optic nerve, the longitudinal outer diameter of the subarachnoid space, the diameter ratio, and the area of the subarachnoid space were determined. RESULTS: In normal subjects, the ring-shaped area of high signal intensity that represented the subarachnoid space was widest behind the globe, then narrowed toward the orbital apex. In patients with papilledema, the area of the subarachnoid space was markedly dilated, the optic nerve was compressed, and the nerve sheath was widened, resulting in a small diameter ratio compared with that of controls. Patients with pallor of the temporal aspect of the optic disc appeared to exhibit dilation of the subarachnoid space; the size of the optic nerve was decreased more than that of the nerve sheath, resulting in a small diameter ratio compared with controls. Patients with complete pallor of the disc, however, exhibited hyperintense optic nerve complexes without a ring-shaped appearance toward the orbital apex. CONCLUSION: Fast spin-echo magnetic resonance imaging appears useful for objectively evaluating the optic nerve and surrounding subarachnoid space in patients with papilledema and optic atrophy.     

Aplasia and hypoplasia of the optic nerve. Comparison of 2 cases

Hypoplasia and aplasia of the optic nerve are congenital anomalies characterized respectively by a marked volume reduction (very small papilla, often identifiable only as a rosy-yellowish area from which the retinal vessels emerge) and by the absence of the optic disk (absence of the nerve and mainly of its vessels) and of the visual functions. These anomalies are often associated with malformations of the central nervous system and of the ocular structures. The defects originate in the embryonal period due to the arrested development of the mesodermal component towards the head of the optic nerve. The aim of this study is to try to interpret the different clinical manifestations of the disease by observing two cases (a female and a male) with a clinical picture of aplasia and hypoplasia of the optic nerve respectively. Both cases presented the following clinical characteristics: developmental defects of the optic nerve, severe microcephaly, positive IgG antibodies against CMV. The association of the optic nerve defect with microcephaly is due to the embryogenic origin of the optic vescicle by prosencephalon. The resulting clinical picture is probably the outcome of an interfering process that the authors have presumed in CMV intrauterine infection, after having excluded the most frequent teratogenic agents (maternal diabetes, tabagism, intake of alcohol ad drugs). Moreover, the different degree of ocular involvement and the different time of onset of the intrauterine pathogenic insult in the two cases could account for the different clinical pictures.     

Bilateral optic disk pallor after unilateral internal carotid artery occlusion

Bilateral pallor of the optic disks was observed in a 52-year-old man after dissection of an internal carotid artery. Diffuse pallor of the ipsilateral optic disk reflected infarction of the ipsilateral optic nerve and "bow-tie" atrophy of the contralateral optic disk reflected infarction of the ipsilateral optic tract. The findings were due to an occlusion of the internal carotid artery proximal to the origin of the ophthalmic artery, resulting also in insufficiency in the area of supply of the anterior choroidal artery.     

Bilateral optic nerve meningioma. Case report

A case of bilateral optic nerve meningioma is reported. The onset of the clinical symptoms, at age 27, resembled unilateral optic neuritis with papilledema, leading to bilateral amaurosis with optic atrophy 4 years later. Skull X-ray revealed a "blistering' type of bone reaction. In the carotid angiogram, the ophthalmic artery appeared quite enlarged and displaced. The CT scan showed a fusiform enlargement of both optic nerves. Pathological diagnosis was based upon an optic nerve biopsy. Including the present case, only 12 instances of bilateral optic nerve meningioma have been reported.     

Leber hereditary optic neuropathy. Electron microscopy and molecular genetic analysis of a case

BACKGROUND: Leber hereditary optic neuropathy (LHON) is a mitochondrial genetic disorder characterized by bilateral central visual loss typically in early adulthood. Few histopathologic studies, including ultrastructural and molecular genetic analysis, have been reported. METHODS: Ocular tissue was obtained postmortem from an 81-year-old woman with LHON from the Queensland 1 pedigree characterized by mutations at nucleotide positions 4160 and 14484. Routine histopathologic studies, electron microscopy, electron-probe analysis, and molecular genetic analysis were performed. RESULTS: Marked atrophy of the nerve fiber and retinal ganglion cell layers and optic nerves was present. Results of electron microscopic examination demonstrated 1.2 microns electron-dense, double-membrane-bound inclusions, consisting of calcium by electron-probe analysis, in retinal ganglion cells. The optic nerve was homoplasmic for mutations 4160 and 14484. CONCLUSION: Optic nerve and inner retinal atrophy in LHON may be a result of metabolic mitochondrial dysfunction leading to intramitochondrial calcification. Homoplasmy for mitochondrial mutations 4160 and 14484 in the leukocyte/platelet fraction of whole blood may correlate with homoplasmy in the optic nerve.     

Deglutition in patients treated with reconstructive laryngectomy]

BACKGROUND: Chronic papilledema may lead to irreversible damage of optic nerve fibers. To preserve visual functions, a decompression of the optic nerve is recommended by means of a fenestration of the optic nerve sheath. In this study long-term results after optic nerve sheath fenestration in patients with idiopathic pseudotumor cerebri are reported. PATIENTS AND METHODS: 14 patients were re-examined 15 to 145 months (mean 62 months) after a fenestration of the retrobulbar optic nerve sheath on 23 eyes by a transconjunctival approach. Surgery was done to treat progressive visual loss or in severe obscurations. The patient's symptoms, visual acuity, visual fields, and ophthalmoscopic findings of the optic nerve head and the central fundus were compared to the preoperative status. RESULTS: Re-examination revealed improvement or stabilisation of objective and subjective findings in 17 eyes, one of them was operated on the more involved contralateral side only. Six eyes showed a recurrence of the papilledema without a functional change for the worse after an interval of 7 to 121 months. Three eyes of two patients ended up with optic atrophy and extensive visual loss. Preoperatively, these eyes had shown cotton wool spots in the optic nerve head and a rapid deterioration of vision. CONCLUSIONS: Fenestration of the retrobulbar optic nerve sheath can prevent further visual loss in most patients with pseudotumor cerebri, unless the eye has already become nearly blind. Postoperatively, ophthalmological controls are necessary at regular intervals because relapses after successful surgery can occur after months or years.     

Acute bilateral amaurosis in sphenoid empyema caused by Streptococcus pneumoniae

PATIENT: A 14-year-old boy suffered from an acute bilateral blindness which occurred in 24-h time, accompanied by headache and raised temperature, with inconspicuous optic nerve head and fundus. After diagnosis of empyema with magnet resonance tomography (MRT) the sphenoid sinus was fenestrated and streptococcus pneumoniae isolated. Liquor and serology being inconspicuous, there was no evidence of leucaemic or autoimmune disease, intoxication or intracranial tumor. CLINICAL COURSE: The condition of the patient improved under systemic antibiotic therapy. The bilateral amaurosis remained and opticus atrophy developed. CONCLUSION: A bilateral amaurosis with descending opticus atrophy as a consequence of a sphenoiditis and spreading inflammation to the meninges and the optic nerve in the area of the chiasm is a rare event. The imaging technique of the MRT offers new opportunities for an early and more pointed diagnosis and therapy.     

The Wolfram syndrome: diabetes mellitus, hypacusis, optic atrophy and short stature in STH deficiency

HISTORY AND CLINICAL FINDINGS: A 43-year-old man was known for 3 years to have diabetes mellitus. For 2 months before admission he had symptoms of hyperglycaemia with polyuria, polydipsia, weight loss, as well as impairment of vision and declining fitness. In addition to bilateral deafness he was clearly of normally proportioned short stature (150 cm). INVESTIGATIONS: The levels of blood sugar (221 mg/dl), HbA1c(10.2%), triglycerides (496 mg/dl) and cholesterol (323 mg/dl) were raised, while the concentration of somatotropic hormone was diminished, both before and after arginine administration. Fundoscopy revealed concentric diminution of the visual fields with left amblyopia. Visual evoked potentials and colour sense testing revealed bilateral optical atrophy, and the audiogram demonstrated deafness. These findings provided the diagnosis of Wolfram syndrome, namely insulin-dependent diabetes mellitus, deafness, optical atrophy and small stature with somatotropic hormone deficiency. TREATMENT AND COURSE: On insulin treatment the metabolic state became normal (HbA1c 7.5%, normal lipid profile). It was decided that the deficiency in somatotropic hormone regulation did not require treatment. CONCLUSION: Cardinal symptoms of the autosomally recessive Wolfram syndrome are insulin-dependent diabetes and optic nerve atrophy. Several types of hormonal abnormalities are associated with it, including a deficiency in the somatotropic axis. To obtain early and adequate hormonal substitution requires extensive endocrinological diagnosis of a disease which frequently becomes manifest in childhood or adolescence.     

Ectopic pregnancy after a laparoscopically-assisted vaginal hysterectomy

PURPOSE/METHODS: Rubinstein-Taybi syndrome is a constellation of clinical findings characterized by mental and motor retardation, broad thumbs and broad first toes, marked growth retardation, microcrania, typical facies, high-arched palate, and cryptorchidism in males. Ocular and adnexal abnormalities are quite common and include antimongoloid slant of the palpebral fissures, epicanthal folds, congenital obstruction of the lacrimal excretory system, ptosis, strabismus, and severe ametropia. Macrocornea, microophthalmos, colobomas of the iris and of the optic nerve head, congenital cataract, and optic nerve atrophy have also been described. Congenital glaucoma is a rare complication. We examined a patient with Rubinstein-Taybi syndrome with bilateral congenital glaucoma. RESULTS/CONCLUSIONS: Examination of this patient revealed bilateral antimongoloid slants of the palpebral fissures, and bilateral congenital glaucoma. Gonioscopic examination revealed the iris to be inserted flatly into the trabecular meshwork. This case emphasizes the importance of detailed, complete ocular examinations in patients with Rubinstein-Taybi syndrome, and also highlights the occurrence of ocular abnormalities rarely associated with this disease.     

Nerve growth factor effects on the song control system of zebra finches

PURPOSE: The authors present the "pattern" visual evoked potentials (VEP) analysis with use of the artificial neural networks (ANN). MATERIALS AND METHODS: The study involved 11 patients with compressive chiasmal optic neuropathy, 20 patients with optic neuritis, 12 patients with anterior ischaemic optic neuropathy, 20 patients with optic nerve atrophy from neuritis, 8 patients with demyelinative neuropathy, 5 patients with oedema optic nerve, 20 healthy persons. The tests of visual evoked potentials were performed with the use of computer system UTAS-E1000. Classification of potentials was made by correlation of outputs of ANN with results of confirmed neuro-ophthalmology conditions. RESULTS: ANN of different architecture were classified correctly in 80-100% of VEP record samples. CONCLUSIONS: The obtained correctness of classification confirms usefulness of VEP analysis as the objective diagnostic method in some neuro-ophthalmological diseases and indicates application of ANN in multifactor analysis.     

Contemporary methods for functional analysis of the optic nerve

Complex methods of statistic analysis for parameters of automated perimetry and visual evoked potentials were presented. The examinations were performed in 14 patients with compressive visual neuropathy, in 14 patients with optic neuritis, in 20 patients with demyelinative visual neuropathy and in 20 patients with optic nerve atrophy. Multidimensional analysis of variance (MANOVA) was performed. The methods proved useful for differential diagnosis of optic neuropathy.