Cases reported "Optic Neuritis"

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1/7. HSV-1--induced acute retinal necrosis syndrome presenting with severe inflammatory orbitopathy, proptosis, and optic nerve involvement.

    OBJECTIVE: To present a unique case in which orbital inflammation, proptosis, and optic neuritis were the initial symptoms of acute retinal necrosis (ARN). The clinical presentation of ARN, as well as the currently recommended diagnostic procedures and guidelines for medical treatment of ARN, are summarized. DESIGN: Interventional case report. TESTING: polymerase chain reaction (PCR) techniques were made on the vitreous for cytomegalovirus, Epstein-Barr virus, herpes simplex virus (HSV), varicella zoster virus, and toxoplasmosis. A full laboratory evaluation was made together with HLA-typing and serologic tests measuring convalescent titers for HSV and other micro-organisms. magnetic resonance imaging scan, computed tomography (CT) scan, and fluorescein angiographic examination were performed. The patient was treated with acyclovir and oral prednisone. MAIN OUTCOME MEASURES: The patient was evaluated for initial and final visual acuity and for degree of proptosis, periocular edema, and vitreitis. RESULTS: The first symptoms and signs of ARN were eye pain, headache, proptosis, and a swollen optic nerve on CT scan. Other than increased c-reactive protein, all blood samples were normal. PCR was positive for HSV-type I in two separate vitreous biopsies. The patient had the strongly ARN-related specificity HLA-DQ7. CONCLUSIONS: This is the first report of HSV-induced ARN presenting with inflammatory orbitopathy and optic neuritis. polymerase chain reaction for HSV-1 was positive more than 4 weeks after debut of symptoms, which is a new finding. The combination of severe vitreitis and retinal whitening, with or without proptosis, should alert the clinician to the possibility of herpes infection and treatment with intravenous acyclovir started promptly.
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2/7. Human herpesvirus 7-associated meningitis and optic neuritis in a patient after allogeneic stem cell transplantation.

    A 9-year-old boy who received allogeneic stem cell transplantation began to vomit from day 10 after transplantation. In addition to vomiting, the patient had a fever (from day 26) and severe headache (from day 34). His cerebrospinal fluid (CSF) (day 41) demonstrated pleocytosis with an absence of leukemic cells. Although the patient's symptoms were resolved with further supportive care, abrupt onset of bilateral decreased vision occurred at day 54. He was diagnosed with bilateral optic neuritis, due to the presence of disc edema and redness. Concomitant with the occurrence of aseptic meningitis, the human herpesvirus 7 (HHV-7) antibody titer increased significantly in this patient. Although neither HHV-6 nor cytomegalovirus (CMV) DNA was detected in CSF collected at day 41, HHV-7 DNA was detected in the sample. Viral DNA was not detected in CSF collected at day 93.
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3/7. Nonfamilial and unusual cases of Leber's hereditary optic neuropathy identified by mitochondrial DNA analysis.

    Peripheral blood mitochondrial DNA (mtDNA) samples from 11 patients with acute optic neuritis or insidious optic atrophy were examined for the mutation at nt 11778 and nt 3460 in polymerase chain reaction products. The mtDNA mutation at nt 11778 was evident in 8 cases, which led to a definite diagnosis of Leber's hereditary optic neuropathy (LHON); 4 of the cases were familial and the remaining 4 cases were nonfamilial. None of the 11 patients showed the nt 3460 mutation. The symptoms and signs were variable among the LHON cases with nt 11778 mutation. Assessment of mtDNA provides a useful diagnostic aid for clinically undefined, seemingly nonfamilial or atypical cases of Leber's hereditary optic neuropathy, particularly in bilateral, insidious optic nerve disease in early childhood.
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4/7. Human herpesvirus-6B active infection associated with relapsing bilateral anterior optic neuritis.

    BACKGROUND AND OBJECTIVES: Human herpesvirus-6 (HHV-6) is the causative agent of exanthem subitum. Both HHV-6 variants, A and B, have been associated with central nervous system (CNS) diseases, suggesting a wide neuropathogenic potential. We describe a case of recurrent bilateral anterior optic neuritis with HHV-6 active infection associated with clinical relapses. CASE REPORT: A 23-year old woman presented with progressive visual impairment, bilateral papillitis and painful ocular movements. Nested polymerase chain reaction (PCR) for dna viruses, HHV-6 variant specific real time quantitative PCR, serological analysis and retrotranscription PCR (RT-PCR) for HHV-6 mRNA transcripts were performed. Nested PCR in PBMC and CSF samples was negative for all viruses but positive for HHV-6 DNA, subtyped as HHV-6B. The disease had a relapsing/remitting course. During relapses PBMC samples remained positive for HHV-6 DNA, and HHV-6 active infection was confirmed by the presence of anti-HHV-6 IgM and of HHV-6 U27 mRNA transcript. High viremia levels and relapses were overlapping. After the last relapse, the patient was successfully treated with gancyclovir. CONCLUSIONS: The case reported here suggests a possible association of HHV-6 in bilateral optic neuritis. HHV-6 could be monitored when bilateral optic neuritis is identified, in order to establish an appropriate antiviral therapy.
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5/7. Varicella-zoster virus retrobulbar optic neuritis in a patient with human immunodeficiency virus.

    PURPOSE: To determine the cause of bilateral retrobulbar optic neuritis followed by progressive outer retinal necrosis in a patient with human immunodeficiency virus (hiv). methods: Extensive ophthalmologic, neurologic, infectious disease, rheumatologic, and radiologic examinations were performed. RESULTS: cerebrospinal fluid samples taken after the onset of bilateral retrobulbar optic neuritis and before the development of clinical progressive outer retinal necrosis disclosed varicella-zoster virus from polymerase chain reaction and viral culture. CONCLUSION: Ophthalmologists and neurologists should consider varicella-zoster virus optic neuritis as a potential precursor of progressive outer retinal necrosis and as a cause of retrobulbar optic neuritis in patients infected with hiv.
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6/7. cerebrospinal fluid myelin basic protein-like material in acute monosymptomatic optic neuritis.

    INTRODUCTION: cerebrospinal fluid (CSF) may provide markers of severity and outcome of optic neuritis. MATERIAL AND methods: We examined the CSF from 29 patients with acute monosymptomatic optic neuritis (AMON) for content of myelin basic protein-like material (MBPLM) and correlated the levels with results of cranial magnetic resonance imaging. The length of the time from onset of AMON to the time of CSF collection did not exceed four weeks. RESULTS: Only two patients (7%), one of whom developed an acute myelopathy one month after AMON, showed an elevated value of CSF MBPLM. No MBPLM was detected in 6 patients (21%), and other 21 (72%) had detectable levels of MBPLM but below the upper limit of normal of 0.1 ng/ml. The value of MBPLM was not significantly correlated with the interval to CSF sampling from onset of AMON or with severity of decreased visual acuity. The highest values of CSF MBLPM were observed among patients with severely decreased visual acuity and among patients with an abnormal MRI (13 of 27 i.e. 48%). CONCLUSIONS: CSF MBPLM was rarely abnormal in AMON. However, CSF MBPLM may have potential value in reflecting disease activity, as the highest values were obtained among patients with CSF sampled soon after the maximum visual dysfunction was reached, with severe visual impairment, and with an abnormal MRI.
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7/7. Paraneoplastic cerebellar syndrome and optic neuritis with anti-CV2 antibodies: clinical response to excision of the primary tumor.

    OBJECTIVE: To describe a patient with a paraneoplastic cerebellar syndrome and optic neuritis with circulating anti-CV2 antibodies and clinical improvement after excision of a small cell lung carcinoma. DESIGN: Report of a case. SETTING: A 62-year-old man simultaneously developed a severe cerebellar syndrome and a bilateral optic neuritis predominantly in the left eye (visual acuity, 20/25 in the right eye; < 20/400 in the left eye; and bilateral swelling of the optic discs). MAIN OUTCOME AND RESULTS: Anti-CV2 antibodies, recently described as associated with paraneoplastic neurological syndrome, were detected in the patient's serum sample. These antibodies were demonstrated to react with the cytoplasm of a subpopulation of oligodendrocytes in the white matter of rat brain in the cerebellum, brainstem, spinal cord, and optic chiasm. The patient was found to have a small cell lung carcinoma, which was removed. After excision of the tumor, the cerebellar syndrome improved dramatically and the papilledema disappeared despite aftereffects of the optic neuritis. CONCLUSIONS: These findings were consistent with the diagnosis of a paraneoplastic neurological syndrome, although both optic neuritis and remission of the cerebellar syndrome are uncommon patterns of paraneoplastic syndromes. CV2 antigen expression by the oligodendrocytes of the cerebellum, brainstem, spinal cord, and optic chiasm correlated with the clinical syndrome observed in our patient. However, the precise pathophysiological role of anti-CV2 antibodies is still unknown.
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