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1/60. Migratory basal ganglia lesions in subacute sclerosing panencephalitis (SSPE): clinical implications of axonal spread.

    We report a boy with subacute sclerosing panencephalitis (SSPE) who exhibited parkinsonian symptoms four months after onset. The symptoms improved after administration of levodopa. One year after onset, bilateral symmetric lesions appeared in the substantia nigra and the putamen, as observed using magnetic resonance imaging. After a one-year interval, the lesions migrated to the bilateral caudate and the cerebellar dentate nuclei. The series of migratory legions, each of which was connected by axonal pathways originating from the substantia nigra, suggests axonal spread of the sspe virus.
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keywords = virus
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2/60. Immunocytochemical characterization of long-term persistent immune activation in human brain after herpes simplex encephalitis.

    The clinical, virological and immunocytochemical features of three children who recovered from acute herpes simplex encephalitis (HSE) before the age of 2 years, and who developed secondary severe focal epilepsy after a symptom-free period, leading to neurosurgery 3-10 years later are described. In one child, relapse of HSE occurred immediately after surgery. In all three patients, brain sample biopsies showed abundant CD3-positive T lymphocytes with a majority of CD8 cells, and abundant activated macrophage-microglial cells, a pattern similar to that found in acute HSE. herpes simplex virus dna was retrieved from the tissue biopsy in one case. The long-term persistent cerebral inflammatory process observed after HSE differed from that observed in another chronic viral disease, subacute sclerosing panencephalitis. This inflammatory reaction may be a result either of low-grade viral expression or self-induced immune activation. The role of inflammation in triggering epilepsy remains hypothetical. Solving these issues should have major therapeutic implications. herpes simplex virus dna latency in brain may be the source of replicative HSE relapse.
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keywords = virus
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3/60. Rapid progressive subacute sclerosing panencephalitis in a 2-year-old child with congenital athyreosis.

    We present the unique case of a 2-year-old girl with congenital athyreosis who acquired primary measles virus infection at the age of 18 months, coincidentally with an Epstein-Barr virus infection. First neurologic symptoms of subacute sclerosing panencephalitis appeared 5 months later, and the girl died within 6 months after a rapid progressive illness. Factors possibly predisposing to this extraordinary disease course-primary measles virus infection at an early age and lack of evidence for immunodeficiency-are discussed.
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ranking = 18748.489151226
keywords = measles virus, virus
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4/60. retinitis and dementia in a pregnant girl: an unusual case.

    A 14-year-old pregnant Caucasian girl presented with a 1-week history of dementia. She had presented 1 year prior with acute unilateral visual impairment and was noted to have macular degeneration of presumed infective aetiology. This evolved to a pigmentary macular lesion. During the course of the current presentation she developed myoclonic jerks. An electroencephalogram revealed periodic spike and slow wave complexes at 1-2 second intervals. blood and cerebrospinal fluid examination showed raised anti-measles IgG antibody titres. Intrathecal synthesis of anti-measles virus antibody was demonstrated unequivocally and a diagnosis of subacute sclerosing panencephalitis was made. A healthy male infant was delivered by elective caesarean section at 33 weeks' gestation. She continued to deteriorate clinically despite treatment with intraventricular alpha-interferon. She had not had primary immunization against measles.
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ranking = 9373.7445756131
keywords = measles virus, virus
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5/60. High-dose intravenous ribavirin therapy for subacute sclerosing panencephalitis.

    Two patients with subacute sclerosing panencephalitis (SSPE) were treated safely and effectively with high doses of intravenous ribavirin combined with intraventricular alpha interferon. The ribavirin concentrations maintained in the serum and cerebrospinal fluid were higher than those which inhibit SSPE virus replication in vitro and in vivo.
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keywords = virus
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6/60. subacute sclerosing panencephalitis.

    subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. patients usually have behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The disease has a gradual progressive course leading to death within 1-3 years. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. patients responding to treatment need to receive it life long. Effective immunisation against measles is the only solution presently available to the problem of this dreaded disease.
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keywords = virus
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7/60. subacute sclerosing panencephalitis (SSPE): isolation of a defective variant of measles virus from brain obtained at autopsy.

    A cytopathic agent causing formation of syncytial giant cells was isolated by co-cultivation of human embryonic lung cells with brain cells obtained at autopsy from a patient with subacute sclerosing panencephalitis. Measles specific intracellular immunofluorescence was detected in syncytial giant cells developed by the agent. Paramyxovirus-like nucleocapsids were observed by electron microscopy in nuclei of the syncytial giant cells. Measles specific immunofluorescence was also detected on the surface of unfixed syncytial giant cells. However, the synycytial giant cells did not produce either virions or hemogglutinin, and did not show hemadsorption of African green monkey red cells. Hence, the newly isolated agent seems to be a defective variant of measles virus, and was designated as the SSPE-"BIKEN" strain.
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ranking = 46869.722878065
keywords = measles virus, virus
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8/60. Spatial and temporal disease progression of adult-onset subacute sclerosing panencephalitis.

    An adult-onset case of subacute sclerosing panencephalitis with occipitofrontal spread of the infection documented clinically and by MRI is reported. autopsy revealed numerous intranuclear viral inclusions and widespread demyelination in both frontal lobes. In the occipital lobes where the disease started 5 years previously, inclusions were rare, but degenerative tissue changes were prominent. This case underlines the importance of measles virus migration for the progression of this fatal disorder.
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ranking = 9373.7445756131
keywords = measles virus, virus
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9/60. Early onset and rapidly progressive subacute sclerosing panencephalitis after congenital measles infection.

    We report an 18-month-old girl with rapidly progressive subacute sclerosing panencephalitis, whose non immunised mother had measles at the time of delivery. The patient presented with repetitive episodes of myoclonic jerks of the head and arms, followed by a drop of head and trunk with frequent falls. EEG, CSF studies and MRI confirmed the diagnosis. Despite therapy with isoprinosine and valproate, seizure activity continued and she became vegetative within 2 months, with severe spasticity and swallowing difficulties, and died at the age of 28 months. Early age of onset and rapid progression were most likely related to haematogenous in utero acquisition of the measles virus prior to delivery, as well as immaturity of neuronal and immune systems. CONCLUSION: this case emphasises the importance of a high measles vaccine coverage in the population in order to prevent the risk of disease in general and, in particular, gestational measles.
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ranking = 9373.7445756131
keywords = measles virus, virus
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10/60. Proton magnetic resonance spectroscopy in a case of subacute sclerosing panencephalitis.

    subacute sclerosing panencephalitis is an encephalopathy caused by a persistent measles virus infection. We examined a 13-year-old girl with subacute sclerosing panencephalitis and performed a magnetic resonance spectroscopic study to evaluate the in vivo pathophysiologic abnormality. The results suggested the occurrence of inflammatory processes and glial proliferation prior to neuronal loss even in magnetic resonance imaging (MRI)-negative regions as well as in MRI-positive regions. The additional resonance peaks were also detected, but further studies should be performed to determine the precise pathophysiologic mechanisms. Novel in vivo modalities such as spectroscopy would be useful as a tool to measure responses to therapy.
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ranking = 9373.7445756131
keywords = measles virus, virus
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