|
Harrison's Principles of Internal Medicine, 17e
| Part Sixteen: Neurologic Disorders > Section 2: Diseases of the Central Nervous System > |
Spectrum of Prion Diseases
Sections: Spectrum of
Prion Diseases, Epidemiology, Pathogenesis, Prion Strains, Species Barrier.
Topics Discussed: creutzfeldt-jakob disease; prion diseases; prions.
Excerpt:
"The sporadic form of CJD is the most common prion disorder in
humans. Sporadic CJD (sCJD) accounts for ~85% of all cases
of human prion disease, while inherited prion diseases account for 10–15% of
all cases (Table 378-2). Familial CJD (fCJD),
Gerstmann-Sträussler-Scheinker (GSS) disease, and fatal
familial insomnia (FFI) are all dominantly inherited prion diseases
that are caused by mutations in the PrP gene.CJD is found throughout the world. The incidence of sCJD is approximately
one case per million population, and thus it accounts for about
one in every 10,000 deaths. Because sCJD is an age-dependent neurodegenerative
disease, its incidence is expected to increase steadily as older
segments of populations in developed and developing countries continue
to expand. Although many geographic clusters of CJD have been reported,
each has been shown to segregate with a PrP gene mutation. Attempts
to identify common exposure to some etiologic agent have been unsuccessful for
both the sporadic and familial cases. Ingestion of scrapie-infected
sheep or goat meat as a cause of CJD in humans has not been demonstrated
by epidemiologic studies, although speculation about this potential
route of inoculation..."
The content above is only an excerpt.
For full access, log into an existing user account below, purchase an annual subscription, or purchase a short-term subscription to the complete website.
|
|
|
|
|