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Страна 1 од 148 резултати
Opsoclonus-myoclonus in an HIV-infected child on antiretroviral therapy--possible immune reconstitution inflammatory syndrome.
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The exact immunopathogenesis and neuroanatomical localization of opsoclonus-myoclonus ataxia syndrome remains unclear. We describe a 1 year 9 month old girl who, shortly after commencement of highly active antiretroviral therapy developed opsoclonus-myoclonus syndrome and subsequently died of
Paraneoplastic opsoclonus myoclonus syndrome associated with inflammatory myofibroblastic tumor in a pediatric patient
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Opsoclonus myoclonus syndrome (OMS) is a rare neurological syndrome caused by a paraneoplastic autoimmune process that affects children with neuroblastic tumors. Treatment includes corticosteroids, intravenous gamma globulin (IVIG), rituximab, and other immunosuppressive therapies. Here, we describe
[A case of chronic inflammatory demyelinating polyradiculoneuropathy with nocturnal myoclonus].
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A 43-year-old man began to notice progressive tingling sensation in his legs and unsteadiness in walking in 1983. His symptoms diminished with prednisolone treatment. In 1986, the same symptoms developed again and abated with prednisolone and plasmapheresis. In 1988 he was admitted because of
Brain inflammation is accompanied by peripheral inflammation in Cstb -/- mice, a model for progressive myoclonus epilepsy.
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Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1) is an autosomal recessively inherited childhood-onset neurodegenerative disorder, characterized by myoclonus, seizures, and ataxia. Mutations in the cystatin B gene (CSTB) underlie EPM1. The CSTB-deficient (Cstb -/- ) mouse model
[Rhythmic, bilateral, synchronous myoclonus with an ascending course. Inflammatory lesions of the spinal cord and brain stem].
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Dementia and myoclonus in a case of cryptococcal encephalitis.
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Dementia and generalized myoclonic jerks were the only neurologic features in a patient with cryptococcal encephalitis. Despite the presence of numerous budding yeasts identified as Cryptococcus neoformans in the CSF, there was no inflammatory reaction. Protein and glucose levels were normal, with
Opsoclonus-myoclonus syndrome and HIV-infection.
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Opsoclonus-myoclonus syndrome (OMS) typically presents with chaotic eye movements and myoclonus with some patients exhibiting ataxia and behavioural disturbance. The pathogenesis may be inflammatory with an infectious or paraneoplastic trigger. In this report, we describe four HIV-infected cases
Opsoclonus-myoclonus-ataxia syndrome in an HIV-infected child.
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Opsoclonus-myoclonus-ataxia (OMA) syndrome typically presents with chaotic eye movements and myoclonus with some patients exhibiting ataxia and behavioural disturbances. The pathogenesis may be inflammatory with an infectious or paraneoplastic trigger. We present a 13-year-old HIV-infected girl who
Pediatric Inflammatory and Autoimmune Neurologic Disorders at a Tertiary Medical Center
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Objectives: To describe the spectrum of pediatric inflammatory neurologic diseases and compare the sensitivity of ancillary testing for these diagnoses.
Methods: We analyzed
Intrathecal immune activation in a case of progressive action myoclonus.
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The case of a patient affected by progressive multifocal myoclonus associated with inflammatory reaction in the cerebrospinal fluid is reported. A multiple sclerosis diagnosis is suggested, even if typical disease course and features are lacking.
Increased prevalence of autoimmune disorders and autoantibodies in parents of children with opsoclonus-myoclonus syndrome (OMS).
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Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disease in childhood which can be associated with neuroblastoma. Since autoantibodies have been detected in some patients with OMS, an autoimmune etiology is suspected. We compared the prevalence of autoimmune disorders and autoantibodies in
Elevated B-cell activating factor BAFF, but not APRIL, correlates with CSF cerebellar autoantibodies in pediatric opsoclonus-myoclonus syndrome.
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Childhood opsoclonus-myoclonus syndrome (OMS) occurs idiopathic or, in association with a neuroblastoma, as a paraneoplastic syndrome. Since autoantibodies were identified in some patients, an autoimmune pathogenesis has been suspected. While the newly discovered B-cell activating factors BAFF and
[Chronic herpes simplex encephalitis initially presenting with persistent myoclonus].
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A 59-year-old female patient with atypical chronic herpes simplex encephalitis was reported. Initial symptom was persistent myoclonus involving the trunk and limb muscles, and later lateral gaze palsy to the left side, cerebellar ataxia, consciousness disturbance and other brainstem symptoms
Neuropathologic findings in idiopathic opsoclonus and myoclonus. Their similarity to those in paraneoplastic cerebellar cortical degeneration.
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The neuropathologic findings in an idiopathic case of the opsoclonus/myoclonus syndrome are reported. Although neurologic dysfunction may have been more widespread, structural lesions were limited to the cerebellum and inferior olives. Severe depletion of Purkinje cells with preservation of granular
Opsoclonus-myoclonus-dysequilibrium syndrome: cytological and immunological dynamics in the serial cerebrospinal fluid in two patients.
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A peculiar clinical presentation characterized by the triad of opsoclonus,myoclonus and ataxia, mainly in a form of dysequilibrium, is usually associated with infectious or paraneoplastic processes. Serial cerebrospinal fluid (CSF) analysis in two patients with opsoclonus-myoclonus-dysequilibrium
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