Infectious cerebral amyloidosis: clinical spectrum, risks and remedies.
The clinical and pathological features of the NIH series of 209 transmitted cases of sporadic Creutzfeldt-Jakob disease (CJD) are compared with familial and iatrogenic forms of spongiform encephalopathy. Real or potential risk factors are noted, including animal sources such as scrapie and BSE, iatrogenic sources such as homografts and cadaveric tissue extracts, and genetic defects in the chromosome 20 amyloid precursor gene. The paper concludes with a discussion of the means by which such risks may be minimized.
Brown
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Laboratory of CNS studies, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD.
D000686:Amyloidosis / Q000453:epidemiology / Q000235:genetics / Q000382:microbiology* / Q000473:pathology; D000818:Animals; D015331:Cohort Studies; D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology / Q000208:ethnology / Q000235:genetics / Q000382:microbiology / Q000473:pathology / Q000635:transmission*; D004340:Drug Contamination; D005260:Female; D005506:Food Contamination; D016098:Gerstmann-Straussler-Scheinker Disease / Q000473:pathology; D006801:Humans; D007049:Iatrogenic Disease; D007729:Kuru / Q000473:pathology; D008297:Male; D008460:Meat / Q000009:adverse effects; D008875:Middle Aged; D009928:Organ Specificity; D010375:Pedigree; D017096:Prion Diseases / Q000517:prevention & control; D012307:Risk Factors; D014180:Transplantation / Q000009:adverse effects; D015047:Zoonoses