TABLE 25.6 Neurodegenerative Diseases Caused by Prions* Disease Description Infectious Diseases Kuru A human disease that was once common in New Guinea. It begins with a loss of coordination, usually followed by dementia. Scrapie A disease of sheep and pigs characterized by intense itching in which the animals tend to scrape themselves against trees, followed by neurodegeneration Mad cow disease Begins with changes in posture and temperament, followed by loss of coordination and neurodegeneration Human Inherlted Dlseases Creutzfeldt-Jakob disease Characterized by loss of coordination and dementia Gerstmann-Straüssler- Scheinker disease Characterized by loss of coordination and dementia Familial fatal insomnia Begins with sleeping and autonomic nervous system disturbances followed by insomnia and dementia "All of these diseases are eventually fatal.

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Some people have a genetic predisposition for developing prion
diseases. Examples are described in Table 25.6. In the case of
Gerstmann-Straüssler-Scheinker disease, the age of onset is
typically 30–50 years, and the duration of the disease (which
leads to death) is about 5 years. Suggest a possible explanation
why someone can live for a relatively long time without
symptoms and then succumb to the disease in a relatively
short time.

TABLE 25.6
Neurodegenerative Diseases Caused by Prions*
Disease
Description
Infectious Diseases
Kuru
A human disease that was once common
in New Guinea. It begins with a loss of
coordination, usually followed by dementia.
Scrapie
A disease of sheep and pigs characterized
by intense itching in which the animals tend
to scrape themselves against trees,
followed by neurodegeneration
Mad cow disease
Begins with changes in posture and
temperament, followed by loss of
coordination and neurodegeneration
Human Inherlted Dlseases
Creutzfeldt-Jakob disease Characterized by loss of coordination and
dementia
Gerstmann-Straüssler-
Scheinker disease
Characterized by loss of coordination
and dementia
Familial fatal insomnia
Begins with sleeping and autonomic
nervous system disturbances followed by
insomnia and dementia
"All of these diseases are eventually fatal.
Transcribed Image Text:TABLE 25.6 Neurodegenerative Diseases Caused by Prions* Disease Description Infectious Diseases Kuru A human disease that was once common in New Guinea. It begins with a loss of coordination, usually followed by dementia. Scrapie A disease of sheep and pigs characterized by intense itching in which the animals tend to scrape themselves against trees, followed by neurodegeneration Mad cow disease Begins with changes in posture and temperament, followed by loss of coordination and neurodegeneration Human Inherlted Dlseases Creutzfeldt-Jakob disease Characterized by loss of coordination and dementia Gerstmann-Straüssler- Scheinker disease Characterized by loss of coordination and dementia Familial fatal insomnia Begins with sleeping and autonomic nervous system disturbances followed by insomnia and dementia "All of these diseases are eventually fatal.
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