Fatal Familial Insomnia

Common Name(s)

Fatal Familial Insomnia

Fatal familial insomnia is a prion disorder showing autosomal dominant inheritance. Prion diseases belong to group of progressive conditions that affect the nervous system in humans and animals.  Fatal familial insomnia is characterized by insomnia with or without a diurnal dreaming state, hallucinations, delirium, and dysautonomia preceding motor and cognitive deterioration. . To date, there is no treatment of the underlying pathological mechanisms of the disease. The signs and symptoms typically begin in adulthood and lead to death within 6 to 32 months.
 

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Condition Specific Organizations

Following organizations serve the condition "Fatal Familial Insomnia" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Fatal Familial Insomnia" returned 15 free, full-text research articles on human participants. First 3 results:

Brain microglia were activated in sporadic CJD but almost unchanged in fatal familial insomnia and G114V genetic CJD.
 

Author(s): Qi Shi, Wu-Ling Xie, Baoyun Zhang, Li-Na Chen, Yin Xu, Ke Wang, Ke Ren, Xiao-Mei Zhang, Cao Chen, Jin Zhang, Xiao-Ping Dong

Journal:

 

Microglial activations have been described in different subtypes of human prion diseases such as sporadic Creutzfeldt-Jakob disease (CJD), variant CJD, Kuru and Gerstmann-Sträussler-Scheinker disease (GSS). However, the situation of microglia in other genetic prion diseases such ...

Last Updated: 19 Jul 2013

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Clinical and familial characteristics of ten chinese patients with fatal family insomnia.
 

Author(s): Qi Shi, Cao Chen, Chen Gao, Chan Tian, Wei Zhou, Baoyun Zhang, Jun Han, Xiao Ping Dong

Journal: Biomed. Environ. Sci.. 2012 Aug;25(4):471-5.

 

Fatal familial insomnia (FFI) is an autosomal dominant prion disease characterized clinically by inattention, sleep loss, dysautonomia, and motor signs. This study is aimed to investigate clinical and familial characteristics of ten Chinese Patients with FFI.

Last Updated: 2 Oct 2012

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Biot's breathing in a woman with fatal familial insomnia: is there a role for noninvasive ventilation?
 

Author(s): Luis Fernando Casas-Méndez, Manel Lujan, Laura Vigil, Gemma Sansa

Journal: J Clin Sleep Med. 2011 Feb;7(1):89-91.

 

We present the case of a 48-year-old woman suffering from fatal familial insomnia (FFI)--a rare prion disease--who developed Biot's breathing and secondary respiratory failure during the early stages of the illness. Once hypercapnia was detected a trial of nocturnal noninvasive ventilation ...

Last Updated: 23 Feb 2011

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Reviews from the PubMed Database

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The terms "Fatal Familial Insomnia" returned 1 free, full-text review articles on human participants. First 3 results:

Self management of fatal familial insomnia. Part 1: what is FFI?
 

Author(s): Joyce Schenkein, Pasquale Montagna

Journal:

 

Fatal familial insomnia (FFI) is a genetically transmitted neurodegenerative prion disease that incurs great suffering and has neither a treatment nor a cure. The clinical literature is devoid of management plans (other than palliative). Part 1 of this article reviews the sparse literature ...

Last Updated: 4 Apr 2007

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