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DeCS
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Descriptor English:
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Prion Diseases
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Descriptor Spanish:
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Enfermedades por Prión
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Descriptor Portuguese:
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Doenças de Príon
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Synonyms English:
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Dementias, Transmissible
Spongiform Encephalopathies, Transmissible
Transmissible Dementias
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Tree Number:
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C10.228.228.800
C10.574.843
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Definition English:
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A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83) |
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Indexing Annotation English:
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GEN or unspecified: prefer specifics
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History Note English:
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1993
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Allowable Qualifiers English:
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Record Number:
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30599
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Unique Identifier:
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D017096
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Occurrence in VHL:
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Similar:
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DeCS SciELO LILACS LIS
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