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Fatal Familial Insomnia

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Fatal Familial Insomnia (FFI) is an extremely rare degenerative brain disease which appears to be genetic in nature.

 


Fatal familial insomnia: Fatal familial insomnia, or FFI, is a very rare inherited disease of the brain. The dominant gene responsible for FFI has been found in just 28 families worldwide.

Fatal familial insomnia (FFI): A hereditary prion disease with disrupted sleep (insomnia), motor abnormalities (myoclonus, ataxia, dysarthria, dysphagia, and pyramidal signs), and hyperactivation of the autonomic nervous system.

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Fatal familial insomnia ... Insomnia
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Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). Kuru was identified in people of an isolated tribe in Papua New Guinea and has now almost disappeared.

Some forms can be inherited such as familial CJD, Gerstmann-Sträussler-Scheinker disease (GSS), and fatal familial insomnia (FFI). The disease is caused by a gene mutation in the prion gene. Other inherited prion diseases are more rare.

Creutzfeldt-Jakob disease (PrP in cerebrum)
Kuru (diffuse PrP deposits in brain)
Fatal Familial Insomnia (PrP in thalamus)
Bovine spongiform encephalopathy (PrP in cerebrum of cows) ...

Creutzfeldt-Jakob Disease (CJD)
Variant Creutzfeldt-Jakob Disease (vCJD)
Gerstmann-Straussler-Scheinker Syndrome
Fatal Familial Insomnia
Kuru ...

Other TSEs include Creutzfeldt-Jakob disease and fatal familial insomnia in humans, bovine spongiform encephalopathy in cattle (also known as mad cow disease), scrapie in sheep and goats, and chronic wasting disease in deer and elk.

who ate the brains of deceased relatives as part of a funerary ritual), scrapie (found in sheep), chronic wasting disease (found in deer), and other rare human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia.

" Besides CJD, there are four other TSEs that affect people: kuru, which was found in an isolated tribe in Papua New Guinea; Gerstmann-Sträussler-Scheinker syndrome (an inherited condition); fatal familial insomnia (an inherited condition); ...

See also: Insomnia, Symptom, Prion, Creutzfeldt-Jakob Disease, Dementia

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