1/177. Progression of myelinated retinal nerve fibers.PURPOSE: To present a case demonstrating progression of retinal nerve fiber myelin and to suggest that myelinated retinal nerve fibers be considered among the peripapillary choristomas. METHOD: Case report. A 46-year-old woman demonstrated progression of myelinated retinal nerve fibers and associated retinal vascular anomalies. RESULTS: Myelinated retinal nerve fibers can be progressive. We confirm earlier reports of vascular anomalies associated with retinal nerve fiber myelin and show that they can also be progressive. CONCLUSIONS: The rarely progressive nature of myelinated retinal nerve fibers and the histopathologic studies published to date suggest that myelinated retinal nerve fibers represent a striking ophthalmoscopic feature resulting from ectopic oligodendrocytes; thus, the entity may be described as an oligodendrocytic choristoma.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/177. The 22-kDa antigen in optic nerve and retinal diseases.OBJECTIVE: patients with unexplained visual loss were evaluated for the possibility of immunologic involvement. Antibody reactions were sought that might identify a common indication of retinal hypersensitivity. methods: The enzyme-linked immunosorbent assay (ELISA) and Western blot analysis were used to identify autoantibody reactions with retina and optic nerve components. Comparisons were made with the autoantibody reaction of normal subjects and patients with recognized forms of retinal decay: macular degeneration, retinitis pigmentosa, diabetic retinopathy, and paraneoplastic retinopathy. RESULTS: Eight patients, one man and seven women, were found to produce an autoantibody reaction with retina and optic nerve, including a novel 22-kDa neuronal antigen present within the retina and optic nerve. One of the eight had retinopathy associated with melanoma (MAR syndrome). Seven of the eight patients had electroretinogram abnormalities, varying from mild to severe. Six displayed features of optic atrophy. One patient with progressive visual loss had visual function stabilized after immunosuppressive therapy. CONCLUSIONS: In the eight cases described, unexplained visual loss was associated with autoantibody reactions with retina and optic nerve, including a common antibody reaction with a 22-kDa neuronal antigen found in the retina and optic nerve. All the patients had either an abnormal electroretinogram or optic atrophy. Six patients had both. The 22-kDa immunologic marker may not be directly involved in the patient's vision loss, but rather may be related to a nonspecific destruction of retina and optic nerve. However, the marker may be useful in identifying a specific subgroup of patients for further analysis.- - - - - - - - - - ranking = 0.90909090909091keywords = nerve (Clic here for more details about this article) |
3/177. Retinal hamartoma in oral-facial-digital syndrome.Only recently have intraocular findings been described in oral-facial-digital syndrome (OFDS), including 5 cases of chorioretinal colobomas and 1 case of optic nerve coloboma. We report a case of a new ocular anomaly associated with this syndrome: a retinal hamartoma in a male infant with OFDS. The patient had bilateral retinal masses that were suspicious for retinoblastoma because of a family history of retinoblastoma. physical examination and imaging studies of the retinal masses could not differentiate between retinoblastoma, hamartoma, or persistent hyperplastic primary vitreous. Subsequent pathologic study of an enucleated globe was diagnostic of a retinal hamartoma. This case further illustrates the heterogeneity of ocular anomalies in OFDS and underscores the importance of a complete ophthalmologic evaluation in patients with this syndrome.- - - - - - - - - - ranking = 0.090909090909091keywords = nerve (Clic here for more details about this article) |
4/177. Punctate outer retinal toxoplasmosis in an hiv-positive child.PURPOSE: To discover whether the outer layer of the retina can be the site for toxoplasmosis in AIDS patients. methods: An hiv-positive child, who previously had a normal ocular examination, was reexamined three months later. This examination showed outer retinal lesions compatible with toxoplasmosis and positive IgM and IgG titers specific for that organism, despite the small drop in the CD4 count. RESULTS: During the first examination, the antibodies for toxoplasmosis were negative. At the three-months follow-up, the anti-toxoplasmosis antibodies were positive and the rest of the workup was negative, suggesting a strong correlation with the patient's fundus pattern. CONCLUSION: We describe a case of punctate outer retinal toxoplasmosis uveitis, which has been previously associated with immunocompetent hosts. We, however, believe that it can be seen in immunocompromised patients as well.- - - - - - - - - - ranking = 0.0013210866543401keywords = organ (Clic here for more details about this article) |
5/177. Demyelination of retinal myelinated nerve fibers in Behcet's disease.A 39 year-old Behcet's patient had demyelination of retinal myelinated nerve fibers after recurrent papillitis and vitritis. Oral prednisolone, cyclosporine, and later periocular corticosteroids and oral colchicine were used but demyelination continued over a 5 year-period. A pseudobulbar palsy with urinary incontinence and pyramidal tract signs developed and azathioprine and corticosteroids were used. Demyelination of retinal nerve fibers stopped and while treatment was underway, the central nervous system (CNS) signs were stable. While the ocular pathology of Behcet's can mirror the CNS signs, indeed optic nerve ischemic demyelination may signal the potential for CNS involvement; azathioprine with prednisone may be more effective in the long-term for optic nerve and CNS involvement than cyclosporine with prednisone.- - - - - - - - - - ranking = 0.72727272727273keywords = nerve (Clic here for more details about this article) |
6/177. Severe juvenile retinoschisis associated with a 33-bps deletion in XLRS1 gene.X-linked juvenile retinoschisis is a form of vitreoretinal dystrophy that is characterized by foveal and peripheral splitting of the retinal nerve fiber layer. Pathognomonic of this disorder is a microcystic radiate appearance in the fovea. We encountered a 10 year-old, mildly retarded, Japanese boy, who exhibited a widely extended macular retinoschisis bilaterally. A break in the inner layer of the left eye mimicked a lamellar macular hole, which is a rare manifestation of the disease. Peripheral retinoschisis was absent. Only a few reports have described marked bilateral macular retinoschisis that involved entire posterior pole, while various other macular findings have been reported. This patient with a severe form of retinoschisis was found to harbor the deletion of 33 base pairs, including the boundary region of exon 3 and intron 3 in the XLRS1 gene.- - - - - - - - - - ranking = 0.090909090909091keywords = nerve (Clic here for more details about this article) |
7/177. Juxtapapillary nerve fiber layer infarction as a complication of coronary artery bypass surgery.BACKGROUND: Recent studies have detailed the prevalence and etiology of ocular complications resulting from coronary artery bypass surgery. Of these, retinal nerve fiber layer infarctions are reported most commonly. The clinical sequelae of nerve infarction may include loss of visual acuity, compromised pupillary function, and visual-field defects (the severity of which may be correlated with the location and extent of the insulted tissue). methods: A patient who had experienced bilateral juxtapapillary nerve fiber layer infarction with subsequent loss of visual acuity and peripheral visual field was followed postoperatively for more than 6 weeks. Immediately before our examination, he underwent quadruple coronary artery bypass graft surgery. RESULTS: From the data collected during initial and follow-up examinations, it was determined that the nerve fiber layer infarction was probably the result of a systemic ischemic event during an otherwise uncomplicated surgery. Such events may include hypovolemic blood loss, systemic hypotension during or following surgery, or a host of complications that would prevent adequate perfusion to capillaries in select regions of the eye. CONCLUSION: The pathology of bilateral juxatapapillary nerve fiber layer infarction as a result of substantial transient systemic ischemia may be explained by examining the microcirculation of this region of the retina. Due to certain anatomic and physiologic characteristics, the capillaries supplying the peripapillary zone are most susceptible to arterial vascular events such as ischemia. It is important to recognize this clinical presentation in order to rule out other possible causes for decreased visual acuity and field defects in the postoperative coronary bypass surgery patient.- - - - - - - - - - ranking = 0.81818181818182keywords = nerve (Clic here for more details about this article) |
8/177. Transpupillary thermotherapy as primary treatment for small choroidal melanomas.OBJECTIVE: To report the short-term follow-up results of eyes containing small choroidal melanomas that were treated with transpupillary thermotherapy. methods: Twenty eyes with suspected small choroidal melanomas were treated with transpupillary thermotherapy using infrared light delivered from the diode laser. RESULTS: The age of the patients ranged from 26 to 82 years. In 14 patients, there was documented growth of the melanoma before transpupillary thermotherapy. The tumor thickness ranged from less than 1.0 to 3.2 mm. Seven tumors were treated more than once. Follow-up ranged from 6 months to more than 3 years. Following treatment, the tumor thickness decreased in all cases, usually within 2 months. Progressive atrophy of tumor mass and loss of pigmentation within the tumor continued beyond 1 year of follow-up in some eyes. Complications included field defects, vascular changes, and macular abnormalities. CONCLUSIONS: Transpupillary thermotherapy of small choroidal melanomas is usually followed by early tumor shrinkage but is complicated by dense scotomas, nerve fiber bundle defects, and, occasionally, macular abnormalities. The short-term follow-up results suggest that transpupillary thermotherapy may arrest the growth of selected small melanomas.- - - - - - - - - - ranking = 0.090909090909091keywords = nerve (Clic here for more details about this article) |
9/177. coffee and doughnut maculopathy: a cause of acute central ring scotomas.AIMS: To report the clinical features of five patients with non-progressive central ring scotomas of acute onset associated with excellent retained visual acuity. methods: Complete neuro-ophthalmological examinations were performed. visual fields were performed by tangent screen, Goldmann, or Humphrey perimetry. In some cases further testing was carried out including fundus photography, fluorescein angiography, ERG, VEP, and neuroimaging. RESULTS: The patients were three women and two men whose ages ranged from 25 to 57 years. Four patients were heavy caffeine consumers while the fifth patient experienced an episode of hypotension. Vision loss was acute in all cases. The onset of vision loss was bilateral/simultaneous in three cases, bilateral/sequential in one case, and unilateral in one case. All affected eyes retained visual acuities of 20/25 or better. Colour vision was subnormal in three of four cases. Visual field defects were characterised by a central ring scotoma having an outer diameter less than 10 degrees. Fundus examination demonstrated temporal optic nerve pallor in three patients (five of 10 affected eyes) and reddish, petaloid macular lesions in one patient. Good visual acuity was maintained for the duration of follow up in all five patients. CONCLUSION: Central ring scotomas with excellent retained visual acuity may present as an acute, bilateral syndrome in patients who are heavy caffeine consumers. The configuration of visual field loss and its location, combined with the presence of temporal pallor in five eyes, suggest that the defect localises to the inner layers of the macula. While these cases could be considered an expansion of the clinical spectrum of acute macular neuroretinopathy, some may represent a distinct entity.- - - - - - - - - - ranking = 0.090909090909091keywords = nerve (Clic here for more details about this article) |
10/177. Acute, idiopathic blind spot enlargement syndrome.An otherwise healthy 12-year-old boy was evaluated for an enlarged blind spot in his left eye. Neither optic nerve edema/neuritis nor a retrobulbar mass explained this finding. Consultation with a neuro-ophthalmologist over a period of 14 months resulted in a diagnosis of acute, idiopathic blind spot enlargement syndrome, a rare and poorly understood ocular condition. To the author's knowledge, this is the youngest case ever reported.- - - - - - - - - - ranking = 0.090909090909091keywords = nerve (Clic here for more details about this article) |
| | Next -> |