RT Book, Section
A1 Prusiner, Stanley B.
A1 Miller, Bruce L.
A2 Kasper, Dennis L.
A2 Fauci, Anthony S.
SR Print(0)
ID 1141412517
T1 PRION DISEASES
T2 Harrison's Infectious Diseases, 3e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9781259835971
LK accessbiomedicalscience.mhmedical.com/content.aspx?aid=1141412517
RD 2024/04/23
AB Prions  are  proteins  that  adopt  an  alternative  conformation,  which  becomes  self-propagating.  Some  prions  cause  degeneration  of  the  central  nervous  system  (CNS).  Once  relegated  to  causing  a  group  of  rare  disorders  of  the  CNS  such  as  Creutzfeldt-Jakob  disease  (CJD),  prions—as  mounting  evidence  shows—also  appear  to  play  a  key  role  in  more  common  illnesses  such  as  Alzheimer’s  disease  (AD)  and  Parkinson’s  disease  (PD).  While  CJD  is  caused  by  the  accumulation  of  PrPSc,  increasing  data  argue  that  Aβ  prions  cause  AD,  α-synuclein  prions  cause  PD,  and  tau  prions  cause  the  frontotemporal  dementias  (FTDs).  In  this  chapter,  we  confine  our  discussion  to  CJD,  which  typically  presents  with  a  rapidly  progressive  dementia  as  well  as  motor  abnormalities.  The  illness  is  relentlessly  progressive  and  generally  causes  death  within  9  months  of  onset.  Most  CJD  patients  are  between  50  and  75  years  of  age;  however,  patients  as  young  as  17  and  as  old  as  83  have  been  recorded.