1/653. Bilateral optic disk edema caused by sarcoidosis mimicking pseudotumor cerebri.PURPOSE: To present a case of retrobulbar optic nerve and chiasm sarcoidosis that mimicked pseudotumor cerebri. methods: A 34-year-old, thin, black woman presented with transient visual obscurations, normal visual acuity, bilateral optic disk edema, and enlarged blind spots. Clinical, medical, and radiologic evaluations were consistent with pseudotumor cerebri. The patient improved while taking acetazolamide, but 6 months later her symptoms worsened. neuroimaging disclosed enhancement of the optic nerve and chiasm. RESULTS: Despite administration of intravenous corticosteroids, the patient's vision worsened. Bilateral optic nerve sheath fenestrations were performed, and pathology disclosed sarcoidosis. CONCLUSION: sarcoidosis of the optic nerves and chiasm may mimic pseudotumor cerebri.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/653. Optic nerve cysticercosis in the optic canal.The authors present a first case of cysticercosis in the optic canal in a fifteen-year-old female patient. cysticercosis of the optic nerve is rare. A cyst in the optic canal, beneath the sheath of the optic nerve, has never been reported. The patient presented with rapidly diminishing vision in the left eye, headache and papillitis. A magnetic resonance imaging revealed a cystic lesion at the entrance of the optic canal. Surgery performed was a transcranial orbitotomy which included deroofing of the optic canal and removal of the cyst from under the sheath of the optic nerve. The cyst proved to be cysticercus histopathologically. The outcome was a remarkable visual recovery.- - - - - - - - - - ranking = 1.75keywords = nerve (Clic here for more details about this article) |
3/653. Cerebral metastasis presenting with altitudinal field defect.A 75-year-old man presented with a unilateral inferior altitudinal visual field defect and a history of weight loss and night sweats. The acuity in the affected eye was 20/200, otherwise his ocular examination was normal. neuroimaging demonstrated a post-fixed chiasm, with a frontal metastasis compressing the intracerebral portion of the optic nerve. A chest x-ray showed classical cannon ball lesions, secondary to malignant melanoma. This is the first case report of an intracerebral tumor producing an inferior altitudinal field defect.- - - - - - - - - - ranking = 0.25keywords = nerve (Clic here for more details about this article) |
4/653. Compression of the visual pathway by anterior cerebral artery aneurysm.Visual failure is an uncommon presenting symptom of an intracranial aneurysm. It is even more uncommon in aneurysms arising from the anterior cerebral artery (ACA). We presented 2 patients with an aneurysm of the A1 segment of the anterior cerebral artery causing visual field defects. One patient presented with a complete homonymous hemianopia due to compression of the optic tract by a giant aneurysm of the proximal left A1 segment. The second patient had an almost complete unilateral anopia caused by compression of the optic nerve and chiasm by an aneurysm of the distal part of the A1 segment with a small chiasmatic hemorrhage and ventricular rupture.- - - - - - - - - - ranking = 0.25keywords = nerve (Clic here for more details about this article) |
5/653. Bilateral periopticointrascleral hemorrhages associated with traumatic child abuse.PURPOSE: To report a case of bilateral periopticoscleral hemorrhages associated with traumatic child abuse. methods: Postmortem gross examination and histopathologic studies of both eyes and the optic nerves of a 6-month-old infant who died from subdural hematoma. RESULTS: Gross examination and histopathologic step sections disclosed bilateral intrascleral hemorrhages around both optic nerves. In addition, bilateral diffuse multilayered retinal, vitreous, and sublaminar (beneath the internal limiting membrane) hemorrhages were present. CONCLUSION: Periopticointrascleral hemorrhages are characteristic of blunt head trauma and may constitute important forensic evidence in cases of suspected child abuse.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
6/653. Inefficiency of the anticoagulant therapy in the regression of the radiation-induced optic neuropathy in Cushing's disease.radiation-induced optic neuropathy is a rare complication (prevalence less than 1%) following radiotherapy of the sellar region. However, the vasculopathy in Cushing's disease predisposes to radiation-induced injury. We report the case of a 24-year-old man with Cushing's disease since he was 16. The hormonal study including bilateral inferior petrosal sinus catheterization diagnosed a pituitary right lesion, but imagiology was always negative. He underwent a transsphenoidal microadenomectomy and the pathological study showed the presence of corticotrophic hyperplasia but no adenoma. Secondary hypothyroidism and hypogonadism as well as permanent diabetes insipidus were diagnosed and because the patient was not cured he underwent a second transsphenoidal total hypophysectomy. After that and because he was still hypercortisolemic, pituitary external irradiation was given in a total dose of 6000 rad. Six months later he developed progressive bilateral visual loss. Cerebral MR revealed focal enhancement of the enlarged optic nerves and chiasm, associated with demyelination areas of the posterior visual pathways. Treatment was tried first with high doses of corticosteroids and later with anticoagulants-heparin EV. 1000 U/h during 7 days followed by warfarin, but unsuccessfully, probably because the patient was already amaurotic at the beginning of the last treatment.- - - - - - - - - - ranking = 0.28008416302352keywords = nerve, disease (Clic here for more details about this article) |
7/653. Optic neuropathy in systemic lupus erythematosus and antiphospholipid syndrome (APS): clinical features, pathogenesis, review of the literature and proposed ophthalmological criteria for APS diagnosis.Optic neuropathy is a well-known ocular manifestation occurring in patients with systemic lupus erythematosus (SLE), and it remains one of the major causes of blindness in these patients. We report data from six SLE patients with optic neuropathy, one of whom was considered to have antiphospholipid syndrome (APS). This patient had monolateral optic neuropathy, whereas the other five SLE patients had bilateral optic nerve disease. We believe that the monolateral occurrence of optic neuropathy in our patient can be considered as a 'focal' neurological disease due to a thrombotic event involving the ciliary vasculature. Conversely, bilateral optic nerve damage in SLE could be considered to be a 'general' neurological disease due to different immunological mechanisms, such as vasculitis. Additionally, the literature on SLE patients affected by optic neuropathy is reviewed to evaluate the major clinical features, particularly neurological features. In reviewing the literature, it appears that bilateral optic neuropathy in SLE occurs more frequently than monolateral optic neuropathy, and the main neurological manifestation seen in these patients is transverse myelitis, particularly in SLE patients with bilateral optic nerve disease. Finally, we propose a clinico-ophthalmological spectrum of APS and outline the ocular clinical manifestations that can be considered as diagnostic for the syndrome.- - - - - - - - - - ranking = 4.7250475069547keywords = nerve disease, nerve, disease (Clic here for more details about this article) |
8/653. Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma.PURPOSE: To determine the frequency of ophthalmic abnormalities in patients with cutaneous T-cell lymphoma (mycosis fungoides and sezary syndrome) and T-cell lymphoma involving the skin and to describe the clinical course of the disease with selected examples. methods: A computerized diagnostic retrieval system was used to identify all patients with T-cell lymphoma involving the skin who were examined at the Mayo Clinic (Rochester, minnesota) between January 1, 1976 and December 31, 1990. The medical records of affected patients were reviewed. RESULTS: During the 15-year interval from 1976 through 1990, cutaneous T-cell lymphoma was diagnosed in 2,155 patients. Of these 2,155 patients, 42 (1.95%; 26 male and 16 female) had at least 1 ophthalmic abnormality attributable to the disease. The diagnoses in these 42 patients were mycosis fungoides in 19, clinical variants of T-cell lymphoma of the skin (most commonly, peripheral T-cell lymphoma) in 11, and sezary syndrome in 12. Cicatricial eyelid ectropion was the most common finding, affecting 17 (40.4%) of the 42 patients. Thirty-seven patients had findings that, although probably not a direct consequence of cutaneous T-cell lymphoma, have been cataloged in previous studies. CONCLUSION: Although ophthalmic abnormalities in patients with cutaneous T-cell lymphoma are relatively uncommon, the manifestations of the disease are diverse and frequently difficult to treat.- - - - - - - - - - ranking = 0.015042081511761keywords = disease (Clic here for more details about this article) |
9/653. Acquired mitochondrial impairment as a cause of optic nerve disease.BACKGROUND: blindness from an optic neuropathy recently occurred as an epidemic affecting 50,000 patients in cuba (CEON) and had clinical features reminiscent of both tobacco-alcohol amblyopia (TAA) and Leber's hereditary optic neuropathy (Leber's; LHON). Selective damage to the papillomacular bundle was characteristic, and many patients also developed a peripheral neuropathy. Identified risk factors included vitamin deficiencies as well as exposure to methanol and cyanide. In all 3 syndromes, there is evidence that singular or combined insults to mitochondrial oxidative phosphorylation are associated with a clinically characteristic optic neuropathy. PURPOSE: First, to test the hypothesis that a common pathophysiologic mechanism involving impairment of mitochondria function and, consequently, axonal transport underlies both genetic optic nerve diseases such as Leber's and acquired toxic and nutritional deficiency optic neuropathies. According to this hypothesis, ATP depletion below a certain threshold leads to a blockage of orthograde axonal transport of mitochondria, which, in turn, leads to total ATP depletion and subsequent cell death. Second, to address several related questions, including (1) How does impaired energy production lead to optic neuropathy, particularly since it seems to relatively spare other metabolically active tissues, such as liver and heart? (2) Within the nervous system, why is the optic nerve, and most particularly the papillomacular bundle, so highly sensitive? Although there have been previous publications on the clinical features of the Cuban epidemic of blindness, the present hypothesis and the subsequent questions have not been previously addressed. methods: patients in cuba with epidemic optic neuropathy were personally evaluated through a comprehensive neuro-ophthalmologic examination. In addition, serum, lymphocytes for dna analysis, cerebrospinal fluid (CSF), sural nerves, and eyes with attached optic nerves were obtained from Cuban patients, as well as from Leber's patients, for study. Finally, we developed an animal model to match the low serum folic acid and high serum formate levels found in the CEON patients, by administering to rats low doses of methanol after several months of a folic acid-deficient diet. Optic nerves and other tissues obtained from these rats were analyzed and compared with those from the Cuban patients. RESULTS: patients from the Cuban epidemic of optic neuropathy with clinical evidence of a selective loss of the papillomacular bundle did much better once their nutritional status was corrected and exposure to toxins ceased. patients with CEON often demonstrated low levels of folic acid and high levels of formate in their blood. Histopathologic studies demonstrated losses of the longest fibers (in the sural nerve) and those of smallest caliber (papillomacular bundle) in the optic nerve, with intra-axonal accumulations just anterior to the lamina cribrosa. Our animal model duplicated the serologic changes (low folic acid, high formate) as well as these histopathologic changes. Furthermore, ultrastructural examination of rat tissues demonstrated mitochondrial changes that further matched those seen on ultrastructural examination of tissues from patients with Leber's. CONCLUSION: mitochondria can be impaired either genetically (as in Leber's) or through acquired insults (such as nutritional or toxic factors). Either may challenge energy production in all cells of the body. While this challenge may be met through certain compensatory mechanisms (such as in the size, shape, or number of the mitochondria), there exists in neurons a threshold which, once passed, leads to catastrophic changes. This threshold may be that point at which mitochondrial derangement leads to such ATP depletion that axonal transport is compromised, and decreased mitochondrial transport results in even further ATP depletion. neurons are singularly dependent on the axonal transport of mitochondria. (- - - - - - - - - - ranking = 12.662548631534keywords = nerve disease, nerve, disease (Clic here for more details about this article) |
10/653. 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 = 2.5200561086823keywords = nerve, disease (Clic here for more details about this article) |
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