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 ?
Any combination of criteria (a) through (e) makes the diagnosis of FFI probable; criterion (f) in combination with any one of the other criteria makes the diagnosis definitive.
The FFI scientists, working together with Cambodian environmentalists, found evidence of species of animals which were not previously thought to inhabit Cambodia, including the fishing cat, Sikkim mouse, the impressed tortoise and woolly horseshoe bat.
A foreign entity that would otherwise be treated as a financial institution and required to register as a participating FFI may be treated as an excepted nonfinancial group entity, and therefore a nonfinancial foreign entity (NFFE), if the entity is a treasury center or holding company (as defined).
The IGA model 1 will involve no direct interaction between the FFI and the IRS except the registration and will involve reporting to local regulators.
The FFI tracks the changes in credit fundamentals across key sectors of the U.
The FFI tracks the changes in credit fundamentals across key sectors of the US economy over the prior quarter and preceding 12 months.
account holders to their government without having to enter into an FFI agreement.
The final regulations provide more streamlined registration and compliance procedures For groups of financial institutions, including commonly managed investment funds and provide additional detail regarding the obligation of FFIs to verify their compliance under FATCA.
for the provision of assistance for tax check, sparing the need of financial institutions to sign FFI agreement with the U.
The Memorandum of Understanding providing the framework for cooperation between FFI and Kumtor was signed in Bishkek in November 2012.
FFI empowers the HSE to charge those who break health and safety laws for related costs, including inspection, investigation and taking enforcement action.
In a nutshell, FFI means that from October 1, HSE inspectors will be able to charge businesses for any work they do requiring the business to correct material breaches of health and safety law.