Due to degeneration of a part of the brain known as thalamus, fatal familial insomnia is said to have occurred. It is also a genetic disorder called the prion disease of the brain resulting in a waxy substance formation called amyloid plaques.


Both the sexes can be affected by this disease and if an individual inherits the mutant gene, chances of suffering are high at some of time. Fatal familial insomnia is found in about 50 families worldwide, the chance of the responsible gene affecting the offspring is 50% if only one parent carries it.



Patients remain completely sleepless and do not respond to treatment or sometimes are untreatable leading to fatal consequences.


When two women from one family died of insomnia in 1974, Italian doctor Ignazio Roiter detected the disease and was confirmed when another member of the same family again fell ill and died of mysterious circumstances ten years later. Many investigations were held in the US where the brain was flown to, and it was found that an insoluble protein called prion was responsible for the disease.


Supposed to strike people in their fifties, the onset of fatal familial insomnia disease can take place even at the age of thirty and risk remain in people till their 60th year. In newborns the disease can be fatal between 7 and 36 months after their birth. Progressing through four stages, the disease shows up when the patient suffers increasing insomnia coupled with panic attacks and phobia lasting four months.


Panic attacks become more pronounced with hallucination for five months. This is followed by rapid weight loss and inability to sleep. At the final stage, the patient turns unresponsive and mute for six months that finally leads to his death.


Fatal familial insomnia is without any cure as scientists keep working on gene therapy to arrive at a solution. But till today there has been no positive result as yet from gene therapy.


For patients stricken by fatal familial insomnia, sleeping pills have no effect. Hope lies to some extent on the symptoms that can be focused on to improve quality of life. Prion related diseases can also cause sudden outbursts of laughter known as laughing disease and researchers have tried to relate fatal familial insomnia related gene disorders to mad cow disease and the chronic wasting disease that plague deer and elk.

Tags: Fatal Consequences, Fatal Familial Insomnia, Fifties, Five Months, Four Months, Gene Therapy, Genetic Disorder, Hallucination, Italian Doctor, Mutant Gene, Mysterious Circumstances, Newborns, Panic Attacks, Plaques, Prion Disease, Rapid Weight Loss, Roiter, Sexes, Sleeping Pills, Waxy Substance