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DeCS
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Descriptor English:
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Subacute Sclerosing Panencephalitis
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Descriptor Spanish:
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Panencefalitis Esclerosante Subaguda
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Descriptor Portuguese:
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Panencefalite Esclerosante Subaguda
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Synonyms English:
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Van Bogaert's Leukoencephalitis
Leukoencephalitis, Subacute Sclerosing
Panencephalitis, Subacute Sclerosing
SSPE
Measles Inclusion Body Encephalitis
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Tree Number:
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C02.182.500.300.600
C02.290.700
C02.782.580.600.500.500.800
C02.839.862
C10.228.228.210.150.300.600
C10.228.228.245.340.700
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Definition English:
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A rare, slowly progressive encephalitis caused by chronic infection with the MEASLES VIRUS. The condition occurs primarily in children and young adults, approximately 2-8 years after the initial infection. A gradual decline in intellectual abilities and behavioral alterations are followed by progressive MYOCLONUS; MUSCLE SPASTICITY; SEIZURES; DEMENTIA; autonomic dysfunction; and ATAXIA. DEATH usually occurs 1-3 years after disease onset. Pathologic features include perivascular cuffing, eosinophilic cytoplasmic inclusions, neurophagia, and fibrous gliosis. It is caused by the SSPE virus, which is a defective variant of MEASLES VIRUS. (From Adams et al., Principles of Neurology, 6th ed, pp767-8) |
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History Note English:
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71; VAN BOGAERT'S LEUKOENCEPHALITIS was see under SUBACUTE SCLEROSING PANENCEPHALITIS 1971-78; was see under CEREBRAL SCLEROSIS, DIFFUSE 1963-70
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Allowable Qualifiers English:
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Record Number:
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13732
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Unique Identifier:
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D013344
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Occurrence in VHL:
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Similar:
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DeCS SciELO LILACS LIS
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