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 17 free, full-text research articles on human participants. First 3 results:

Does the clinical phenotype of fatal familial insomnia depend on PRNP codon 129 methionine-valine polymorphism?
 

Author(s): Sven Rupprecht, Alexander Grimm, Torsten Schultze, Jan Zinke, Panagiota Karvouniari, Hubertus Axer, Otto W Witte, Matthias Schwab

Journal:

 

Fatal familial insomnia (FFI) is a rare, hereditary prion-protein disease. Methionine-valine polymorphism at codon 129 of the prion-protein gene (PRNP) determines the phenotype in other hereditary prion-protein diseases, but association with the clinical phenotype in FFI remains uncertain. ...

Last Updated: 16 Dec 2013

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A proposal of new diagnostic pathway for fatal familial insomnia.
 

Author(s): A Krasnianski, P Sanchez Juan, Claudia Ponto, M Bartl, U Heinemann, D Varges, W J Schulz-Schaeffer, H A Kretzschmar, I Zerr

Journal: J. Neurol. Neurosurg. Psychiatr.. 2014 Jun;85(6):654-9.

 

In absence of a positive family history, the diagnosis of fatal familial insomnia (FFI) might be difficult because of atypical clinical features and low sensitivity of diagnostic tests. FFI patients usually do not fulfil the established classification criteria for Creutzfeldt-Jakob ...

Last Updated: 7 May 2014

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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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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
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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