Search on: DEMENTIAS, TRANSMISSIBLE 
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Descriptor English:   Prion Diseases 
Descriptor Spanish:   Enfermedades por Prión 
Descriptor Portuguese:   Doenças de Príon 
Synonyms English:   Dementias, Transmissible
Spongiform Encephalopathies, Transmissible
Transmissible Dementias  
Tree Number:   C10.228.228.800
C10.574.843
Definition English:   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) 
Indexing Annotation English:   GEN or unspecified: prefer specifics
History Note English:   1993 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications CN congenital
DI diagnosis DH diet therapy
DT drug therapy EC economics
EM embryology EN enzymology
EP epidemiology EH ethnology
ET etiology GE genetics
HI history IM immunology
ME metabolism MI microbiology
MO mortality NU nursing
PS parasitology PA pathology
PP physiopathology PC prevention & control
PX psychology RA radiography
RI radionuclide imaging RT radiotherapy
RH rehabilitation SU surgery
TH therapy TM transmission
US ultrasonography UR urine
VE veterinary VI virology
Record Number:   30599 
Unique Identifier:   D017096 

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