Search on: SPONGIFORM ENCEPHALOPATHIES, TRANSMISSIBLE 
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Descriptor English:   Prion Diseases 
Descriptor Spanish:   Enfermedades por Prión 
Descriptor Portuguese:   Doenças Priônicas 
Synonyms English:   Dementia, Transmissible
Dementias, Transmissible
Disorder, Prion-Induced
Disorders, Prion-Induced
Encephalopathies, Spongiform, Transmissible
Encephalopathies, Transmissible Spongiform
Encephalopathy, Transmissible Spongiform
Human Transmissible Spongiform Encephalopathies, Inherited
Inherited Human Transmissible Spongiform Encephalopathies
Prion Disease
Prion Induced Disorder
Prion Protein Disease
Prion Protein Diseases
Prion-Associated Disorders
Prion-Induced Disorder
Prion-Induced Disorders
Spongiform Encephalopathies, Transmissible
Spongiform Encephalopathy, Transmissible
Transmissible Dementia
Transmissible Dementias
Transmissible Spongiform Encephalopathies
Transmissible Spongiform Encephalopathy  
Tree Number:   C01.207.800
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 DG diagnostic imaging
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
RT radiotherapy RH rehabilitation
SU surgery TH therapy
TM transmission UR urine
VE veterinary VI virology
Record Number:   30599 
Unique Identifier:   D017096 

Occurrence in VHL: 		 

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