fatal familial insomnia

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insomnia

 [in-som´ne-ah]
abnormal wakefulness; a sleep disorder consisting of an inability to fall asleep easily or to remain asleep throughout the night. The frequency of persistent insomnia is high; epidemiologic data indicate that it is the most common sleep disorder in the industrialized world. The causes may be physical, psychological, psychiatric, or presence of a specific sleep disorder. adj., adj insom´niac. 

The American Academy of Sleep Medicine recommends that health care practitioners should screen all patients for symptoms of insomnia during health examinations. Fatigue, irritability, reduction in memory, and loss of ability to concentrate are among the daytime manifestations of insomnia.

The treatment of insomnia must be individualized, based on the underlying cause. Physical and mental health problems must be addressed, although they cannot always be successfully treated. Specific medications for sleep, such as sedatives, hypnotics, and other agents are frequently used but are often asociated with development of tolerance, or with rebound insomnia when they are discontinued. Nonpharmacologic treatments that have strong research support include the following: stimulus control to retrain the person who is unable to sleep so that he or she re-associates the bed and bedroom with sleep; progressive muscle relaxation; paradoxical intention therapy where the patient stays awake to eliminate performance anxiety related to sleep; biofeedback; and multi-component (cognitive) therapy.

Numerous papers and guidelines to support evidence-based practice in the management of insomnia are available by writing to the American Academy of Sleep Medicine, 6301 Bendel Road NW, Suite 101, Rochester, MN 55901 or looking at their web site at http://www.aasmnet.org/practiceparameters.htm.
fatal familial insomnia an inherited prion disease, transmitted as an autosomal dominant trait. The cause is unknown, but it seems to affect primarily the thalamus with disruptions in the sleep-wake cycle. Onset is typically in midlife, characterized by progressive insomnia, hallucinations, and motor abnormalities followed by stupor and coma ending in death within 6 months to 3 years of onset. There may also be excessive sweating, elevated body temperature and blood pressure, and tachycardia.
primary insomnia a dyssomnia characterized by persistent difficulty initiating or maintaining sleep or by persistently nonrefreshing sleep, but not due to any other psychological or physical condition.

fatal familial insomnia

[MIM*600072] an autosomal dominant progressive neuropathy with progressive insomnia and thalamic lesions.

fa·tal fa·mil·i·al in·som·ni·a

(fātăl fă-milē-ăl in-somnē-ă)
[MIM*600072] A progressive neuropathy with worsening sleeplessness and thalamic lesions.

fatal familial insomnia

A genetically-determined condition occurring between the ages of 40 and 60, involving progressively worsening insomnia, intolerance to heat, watering eyes, progressive difficulty in walking, memory deterioration, speech defect, muscle jerks, progressive physical and mental deterioration and death within 7 to 33 months of onset. The disease, which features severe loss of nerve fibres in the thalamic nuclei at the base of the brain, is one of the growing list of serious disorders caused by abnormal prion proteins—a list that also includes Creutzfeldt-Jakob disease, new variant CJD, kuru, and Gerstmann-Sträussler disease. Sporadic fatal insomnia is also a prion disease and is probably identical to fatal familial insomnia.
References in periodicals archive ?
However, we failed to calculate frailty in 1.0% (n=8) and 6.0% (n=57) of the elderly for FRAIL and FFI, respectively, because of missing data.
FFI proposes that at least two members of the film fraternity be incorporated in the Board so that they can explain the process of using animals and also take into cognizance the care and difficulties.
The distinction that Patsy Lightbown and I made between integrated and isolated FFI (Spada & Lightbown, 2008) is as follows.
FFI chief executive Johnny Tomazos said the group was eager to expand its presence in the Bahraini market and looked forward to introducing Clay within such a premium location.
The pathogenesis of FFI is considered to be due to the loss of the natural function of the PrP.
The reporting obligation has posed legal obstacles, the most contentious of which is the legality of FFIs disclosing the required information in possible violation of domestic laws, such as those on data protection rules and confidentiality issues.
FFI's current documentary collaboration with IMAX and Warner Brothers, "Pandas," has now been launched in theatres in the US and has been met with critical acclaim.
Post, "The Foot Function Index with verbal rating scales (FFI-5pt): A clinimetric evaluation and comparison with the original FFI," The Journal of Rheumatology, vol.
Through completion of the certificate program, Pilar has gained a deeper understanding of the needs of family-owned enterprises and the many roles family business and non-family members play, says Judy Green, FFI president.
The trend in potential drivers or constraints on economic growth or decline is indicated by the relative strength or weakness of the FFI, which ranges from +10 to -10.