1/102. Extensive brain calcification in two children with bilateral Coats' disease.We report two children with bilateral Coats' disease associated with cerebral calcifications in the basal ganglia and deep white matter, asymptomatic at the time of their discovery. cerebellar ataxia developed secondarily in one of them. Both children were born small for date and had febrile convulsive seizures. Three similar patients have been previously reported, two of them in the same sibship; the third reported patient died of aplastic anemia. Bilateral Coats' disease in children should prompt systematic CT scan in search of cerebral calcifications. If present, neurological and genetic prognosis should be cautious.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/102. 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 = 2.1490591626255keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/102. Cerebroretinal vasculopathy mimicking a brain tumor: a case of a rare hereditary syndrome.We report a 35-year-old man with hereditary cerebroretinal vasculopathy (CRV) characterized by retinal microvascular changes and a right frontal intracerebral mass lesion that suggested a brain tumor. Histopathologic analysis of the patient's brain lesion as well as reviewed specimens of the patient's mother, who had reportedly died of a brain tumor, showed no neoplasia but did show cerebral microvasculopathy. CRV should be considered as a differential diagnosis for patients with intracerebral mass lesions, retinal vascular changes, and a positive family history of "brain tumors."- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
4/102. Selective loss of the electroretinogram B-wave in a patient with Creutzfeldt-Jakob disease.Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disease characterized by movement abnormalities and dementia that inevitably progress to death. Familial, infectious, and sporadic forms of the disease are recognized. The worldwide incidence of CJD is estimated at 1:1,000,000 per year, and it affects middle-aged men and women in roughly equal proportions. The disease is caused by a unique infectious vector, the prion, which is a mutant form of a normally occurring cell surface protein found predominantly in the central nervous system. A significant proportion of patients with CJD will have visual disturbances at some point in their illness and may therefore consult a neuro-ophthalmologist. The case of a woman in whom the diagnosis of CJD was not known until autopsy is reported. Early in the course of her disease, she sought ophthalmic consultation because of vision problems.- - - - - - - - - - ranking = 2.1490591626255keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/102. Ocular histopathologic study of a patient with the T 8993-G point mutation in Leigh's syndrome.OBJECTIVE: To report the histopathologic findings of eyes from a patient with Leigh's syndrome associated with the T 8993-G point mutation in mitochondrial dna (mtDNA). DESIGN: Case report. INTERVENTION: A child with hypotonia, developmental delay, persistent lactic acidosis, seizures, and ataxia died of aspiration pneumonia at 15 months of age. Analysis of dna isolated from blood was positive for the T to G point mutation at position 8993 in mtDNA, and the proportion of mutant genomes was estimated at approximately 95%. The type and distribution of abnormalities seen in the brain at autopsy were consistent with those in patients with Leigh's syndrome. MAIN OUTCOME MEASURES: The left eye was examined by light microscopy, and segments of the right eye were examined by transmission electron microscopy. Genetic analysis on dna isolated from blood was performed. RESULTS: Thinning of the nerve fiber and ganglion cell layers was present in the nasal aspect of the macula, and mild atrophy of the temporal aspect of the optic nerve head and optic nerve was present. Electron microscopic study disclosed numerous distended mitochondria in all cells, but particularly in the retinal pigment epithelium, nonpigmented ciliary epithelium, and corneal endothelium. CONCLUSION: This is a report of the ocular histopathologic findings in Leigh's syndrome with the T 8993-G point mutation. The light microscopic findings were similar to those of patients with similar features reported previously. In addition, ultrastructural abnormalities of mitochondria were present.- - - - - - - - - - ranking = 0.25keywords = brain (Clic here for more details about this article) |
6/102. Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature.OBJECTIVE: The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature. MAIN OUTCOME MEASURES: A review of nine patients with AMPPE and CNS involvement was performed. charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. RESULTS: Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, with an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. visual acuity was variable and ranged from 20/20 to count fingers. The spectrum of CNS findings ranged from headaches to sagittal sinus thrombosis. CONCLUSIONS: Acute multifocal placoid pigment epitheliopathy can be associated with CNS abnormalities and permanent visual deficits. neuroimaging, lumbar puncture, and cerebral angiography analysis provide useful diagnostic tools when CNS involvement is suspected. Intravenous corticosteroids and collaboration with neurovascular colleagues should be considered in these situations. In cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids.- - - - - - - - - - ranking = 10.745295813128keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/102. Presumed Eales' disease with neurologic involvement: report of three cases.PURPOSE: To report three cases of presumed Eales' disease with neurologic lesions. methods: case reports, systemic and neurologic evaluation, and magnetic resonance imaging. RESULTS: All three patients were young men who had seizures in the past; two had migrainous headache. magnetic resonance imaging showed putaminal infarct in two cases and edema in the white matter of temporal cortex was noticed in one case. Clinical features in all these patients were suggestive of Eales' disease. CONCLUSION: Ischemic infarction of the brain can be seen in clinically suspected cases of Eales' disease.- - - - - - - - - - ranking = 0.25keywords = brain (Clic here for more details about this article) |
8/102. Evolution of sarcoid granulomas of the retina.PURPOSE: To report a case of a young woman with a history of sarcoidosis who developed retinal granulomas. methods: Case report. RESULTS: A 33-year old woman with history of sarcoidosis with involvement of the central nervous system, confirmed by skin biopsy, bronchoscopy, and neuroimaging, presented with visual loss and was found to have choroidal and optic nerve granulomas in the left eye, and subsequently developed retinal granulomas in the left eye. CONCLUSION: Retinal granuloma is a rare manifestation of sarcoidosis.- - - - - - - - - - ranking = 2.1490591626255keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/102. Subretinal cysticercosis.BACKGROUND: [corrected] cysticercosis is a parasitic infestation of different body organs by cysticercosis cellulosae, a larval form of the helminth, taenia solium, known commonly as pork tapeworm. Ocular involvement of cysticercosis is most common. Other sites of infestation include the central nervous system, subcutaneous tissue, skeletal muscles, and heart muscle. patients with ocular cysticercosis may be asymptomatic or suffer mild to severe vision loss. CASE REPORT: A patient presented with painless vision loss secondary to a parasitic infection by presumed subretinal cysticercosis. The clinical appearance, differential diagnosis, and management of subretinal cysticercosis are discussed. CONCLUSION: Prompt diagnosis and referral to a retinal surgeon are essential for the successful treatment of this condition.- - - - - - - - - - ranking = 2.1490591626255keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/102. Retinopathy associated with high-dose interferon alfa-2b therapy.PURPOSE: To present seven patients who developed retinopathy while receiving high-dose interferon alfa-2b therapy for adjuvant treatment of high-risk melanoma. methods: Retrospective case series. RESULTS: Seven patients developed a retinopathy while receiving high-dose interferon alfa-2b therapy for adjuvant treatment of high-risk melanoma. Five patients had normal visual acuity, but retinopathy with cotton- wool spots and/or retinal hemorrhages with the retinopathy resolved after stopping treatment after detection. Two patients developed severe retinopathy with vision loss to counting fingers and hand motions without resolution of the retinopathy. The duration of the maintenance treatment before detection of the retinopathy for all patients ranged from 6 to 26 weeks. The total dose received at time of detection of retinopathy ranged from 816 to 1770 million units. Confounding factors included hypertension, thrombocytopenia, anemia, and a history of prior chemotherapy in one patient. Also, one patient received an investigational ganglioside vaccine, one had a history of radiation treatment to the brain, and six received paroxetine. CONCLUSIONS: patients receiving interferon alfa-2b are at risk for developing an associated retinopathy. The risk appears to be greater with higher dosage therapy. In addition, severe vision loss can be seen with interferon alfa-2b-associated retinopathy. The effect of treatment with selective serotonin reuptake inhibitors, such as paroxetine, in increasing the incidence of this complication is unknown. patients need to be monitored until the retinopathy is resolved to screen for sequelae, including retinal neovascularization.- - - - - - - - - - ranking = 0.25keywords = brain (Clic here for more details about this article) |
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