RT Book, Section
A1 Prusiner, Stanley B.
A1 Miller, Bruce L.
A2 Jameson, J. Larry
A2 Fauci, Anthony S.
A2 Kasper, Dennis L.
A2 Hauser, Stephen L.
A2 Longo, Dan L.
A2 Loscalzo, Joseph
SR Print(0)
ID 1184194039
T1 Prion Diseases
T2 Harrison's Principles of Internal Medicine, 20e
YR 2018
FD 2018
PB McGraw-Hill Education
PP New York, NY
SN 9781259644016
LK accesspharmacy.mhmedical.com/content.aspx?aid=1184194039
RD 2024/04/20
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  also  appear  to  play  a  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  prions,  recent  investigations  demonstrate  unequivocally  that  α-synuclein  prions  cause  multiple  system  atrophy  (MSA).  Infectious  MSA  prions  have  been  recovered  from  human  brain  samples  stored  in  formalin  for  up  to  20  years.  Similar  resistance  to  formalin  was  demonstrated  for  brain  samples  from  sheep  with  scrapie.  Increasing  data  argue  that  Aβ  prions  contribute  to  AD,  α-synuclein  prions  to  PD,  and  tau  prions  to  some  types  of  frontotemporal  dementia  (FTD).  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.  The  role  of  prions  in  the  pathogenesis  of  neurodegenerative  diseases  is  reviewed  in  Chap.  417.