11/113. Leber hereditary optic neuropathy, progressive visual loss, and multiple-sclerosis-like symptoms.PURPOSE: To report a case of Leber hereditary optic neuropathy with multiple-sclerosis-like symptoms. methods: Observational case report. A 34-year-old man was found to have Leber hereditary optic neuropathy and a mutation at position 11778 of the mitochondrial genome. The progression of vision loss and onset of weakness in the right leg warranted neuroimaging. RESULTS: magnetic resonance imaging documented multiple lesions in the brain and spinal cord. CONCLUSION: Although rarely reported, progression of optic neuropathy over months has been previously documented in Leber hereditary optic neuropathy. The emergence of multiple sclerosis-like symptoms and signs in our patient may be part of the spectrum of Leber hereditary optic neuropathy or a coincidental occurrence.- - - - - - - - - - ranking = 1keywords = optic (Clic here for more details about this article) |
12/113. neuromyelitis optica following infectious mononucleosis.A case of moderatley severe neuromyelitis optica following infectious mononucleosis is described as the first reported instance of this complication. Total recovery occurred. Rapid improvement followed the commencement of corticosteroids. It is postulated that the pathological process was one of postinfectious demyelination.- - - - - - - - - - ranking = 0.55555555555556keywords = optic (Clic here for more details about this article) |
13/113. Visual recovery in a man with the rare combination of mtDNA 11778 LHON mutation and a MS-like disease after mitoxantrone therapy.We describe a young man with prognostic unfavourable homoplasmatic mitochondrial dna(mt dna) 11778 Leber's hereditary optic neuropathy (LHON) point mutation and confirmed multiple sclerosis (MS). This combination of LHON and MS-like disease is rare in both sexes, and in men has been described in only a few case reports. In a 4-year follow-up during immunosuppressive therapy with mitoxantrone, we found a remarkable time delayed visual recovery 12 months after acute onset of rapid sequential bilateral subtotal visual loss followed by episodes of isolated acute demyelinative optic neuropathy. Visual recovery to such extent after this latency is uncommon in both mtDNA 11778 LHON mutation and optic neuritis (ON) in MS. Relapses in visual deterioration must be considered as extremely rare in LHON. This case might support the hypothesis of an immunological pathogenetic factor in combined LHON and MS, and possibly in LHON alone. We suggest a search for the LHON mutation in MS patients with predominant visual impairment, independent of patients' gender.- - - - - - - - - - ranking = 31.160492922521keywords = optic neuritis, neuritis, optic (Clic here for more details about this article) |
14/113. Familial multiple sclerosis: case study of three affected siblings.We report on three sisters with new-onset multiple sclerosis (MS). The symptoms of the eldest sister began in 1993 with lower-limb weakness and paraesthesia. In 1998, she had limb weakness, nystagmus and ataxia. magnetic resonance imaging (MRI) of the brain, the cerebrospinal fluid (CSF) examinations, and evoked potentials verified MS. The middle sister exhibited left-side optic neuritis in 1998. All findings pointed to MS. The third sister had subjective complaints such as paraesthesias and vertigo. MRI and CSF results supported the diagnosis. Both parents and all four grandparents are without neurological signs; the brain MRI examinations on the parents were negative. The prevalence of familial MS in first-degree relatives is 5-10%, while that in twins is 20-30%. In this case, environmental factors seem to play the crucial role. Although the anamnesis as concerns MS proved negative in the other family members examined here, further genetic examination of the sisters is needed.- - - - - - - - - - ranking = 30.938270700299keywords = optic neuritis, neuritis, optic (Clic here for more details about this article) |
15/113. An unusual junctional scotoma.A 28-year-old woman presented with painful unilateral left visual loss, impaired color vision, left afferent pupillary defect, and normal ocular fundus. Although optic neuritis was first suspected, visual fields disclosed a junctional scotoma related to chiasmal demyelination, due to a probable multiple sclerosis.- - - - - - - - - - ranking = 30.938270700299keywords = optic neuritis, neuritis, optic (Clic here for more details about this article) |
16/113. Eales' disease with neurological involvement. Part 2. pathology and pathogenesis.Detailed neuropathologic examination was carried out on 1 case of Eales' disease with CNS involvement, in the form of retinal vasculopathy, followed first by signs of brain stem and cerebellar disease and then by a myelopathy, with death 4 years later from retinal infection. There was mild chronic inflammation in the retina, and sub-total demyelination of one optic nerve. The brain stem and cerebellum showed extensive vasculopathy, with various stages of venous change extending from proliferation and dilatation to haemorrhage, or to thickening with hyalinisation. The perivenular brain tissue, particularly of the cerebellum, often showed demyelination, with relative axon preservation, but no inflammation. Similar, but less pronounced venopathy was seen in the dorsal cord. There was ascending degeneration of Goll's columns and descending degeneration of the lateral columns.- - - - - - - - - - ranking = 0.11111111111111keywords = optic (Clic here for more details about this article) |
17/113. Progressive outer retinal necrosis in immunocompetent patients treated initially for optic neuropathy with systemic corticosteroids.PURPOSE: To report two cases of progressive outer retinal necrosis occurring in immunocompetent individuals after treatment with corticosteroids for presumed optic neuropathy. DESIGN: Observational case report. SETTING: University-based tertiary eye hospital. methods: Retrospective review of existing clinical records. RESULTS: Two patients were treated empirically with systemic corticosteroids for suspected inflammatory papillopathy. Subsequently, both were diagnosed with necrotizing herpetic retinitis with features of progressive outer retinal necrosis. anterior chamber paracentesis confirmed varicella-zoster infection. Both patients were human immunodeficiency virus negative; one patient with rheumatoid arthritis was taking etanercept. Both became completely blind in one eye despite intensive treatment with antiviral medication intravenously and intravitreally. CONCLUSIONS: Progressive outer retinal necrosis is not confined to patients with underlying severe immunodeficiency, such as acquired immune deficiency syndrome. Initial treatment of acute, unexplained vision loss with systemic corticosteroids may lead to catastrophic visual loss in patients with evolving necrotizing herpetic retinopathy.- - - - - - - - - - ranking = 0.55555555555556keywords = optic (Clic here for more details about this article) |
18/113. multiple sclerosis-like disease secondary to alpha interferon.PURPOSE: To describe bilateral optic neuritis that occurred as an adverse effect of recombinant and natural interferon alpha administration. methods: Report of two cases. Case 1, a 62-year-old woman, developed bilateral optic neuritis with decreased sensation of vibration and increased deep tendon reflex in the lower extremities after a seven-month use of recombinant interferon alpha-2a for chronic active hepatitis c. Case 2, a 29-year-old woman, developed bilateral optic neuritis combined with numbness of the lower extremities as well as bowel and bladder dysfunction after a 22-month use of recombinant interferon alpha-2b for chronic myelogenous leukemia. After a two-month interruption of interferon administration, natural interferon alpha was given but followed by another episode of the same neurological manifestations. RESULTS: In both cases, magnetic resonance imaging demonstrated multiple small high-intensity areas in the cerebral white matter and spinal cord, while cerebrospinal fluid examination disclosed mononuclear cell increase and protein elevation including myelin basic protein, all of which simulated the features of multiple sclerosis. The two patients underwent several courses of pulse corticosteroid therapy, each course consisting of three days of methylprednisolone 1000 mg daily, resulting in visual recovery to some extent. CONCLUSIONS: optic neuritis in combination with other neurological signs, simulating multiple sclerosis, should be included in the list of adverse effects of recombinant and natural interferon alpha administration.- - - - - - - - - - ranking = 104.22797513638keywords = optic neuritis, neuritis, optic (Clic here for more details about this article) |
19/113. Autoimmune optic neuropathy with anticardiolipin antibody mimicking multiple sclerosis in a child.PURPOSE: To demonstrate that autoimmune optic neuropathy (AON) may occur in the pediatric population. DESIGN: Interventional case report. methods: A 4-year-old developed four episodes of bilateral optic neuritis with mild concurrent weakness, ataxia, or dizziness; one episode of unilateral optic neuritis; and one episode of weakness over a period of 4 years. RESULTS: Autoimmune optic neuropathy was diagnosed because of the presence of anticardiolipin antibody and an abnormal skin biopsy with thrombin and immunoreactant deposition. She was treated with corticosteroids, aspirin, and gammaglobulin. This diminished the frequency and intensity of her attacks. CONCLUSION: This case represents the diagnosis and treatment of AON in a child.- - - - - - - - - - ranking = 62.543208067264keywords = optic neuritis, neuritis, optic (Clic here for more details about this article) |
20/113. Occurrence of a multiple sclerosis-like illness in women who have a Leber's hereditary optic neuropathy mitochondrial dna mutation.Eight women are described who presented with bilateral, usually sequential, optic neuropathy, six of whom later developed a neurological syndrome indistinguishable from multiple sclerosis (MS). magnetic resonance imaging, performed in five of the patients with an MS-like illness and in the two others with optic neuropathy alone, showed widespread white matter lesions as seen in MS. All of these women had matrilineal relatives with Leber's hereditary optic neuropathy, although this was not always apparent at presentation, and the most common mitochondrial dna mutation associated with this disorder was detected in each of the women and their affected relatives. On the basis of observations made in these patients, the clinical features of Leber's hereditary optic neuropathy in males, and evidence for mitochondrially encoded peptides involved in the immune response in rodents, we propose that optic nerve damage in this disease could be immunologically mediated and that mitochondrial genes may contribute to susceptibility to MS.- - - - - - - - - - ranking = 1keywords = optic (Clic here for more details about this article) |
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