Freaky Fatal Familial Insomnia

Fatal Insomnia (2010)A reader inspired me to research FFI. It’s a natural follow-up to my blog about the bogus Russian Sleep Experiment of the 1940s, except this tragic disorder is real. FFI is a bizarre syndrome. It is almost always caused by a genetically inherited mutation to a protein called PrPC, (don’t ask me), but can develop in patients with a non-inherited mutation called sporadic fatal insomnia (sFI). How long it takes scientists to figure this stuff out, I’ll never know. They must lose sleep in the process.

FFI is incurable and involves worsening insomnia that is so severe in nature it leads to hallucinations, delirium, and dementia. The average survival span for patients diagnosed with FFI after the onset of symptoms is 18 months. Imagine receiving that diagnosis in the middle of your hallucinations and delirium? These poor people must live in terror for a year and a half. Perhaps death is a relief.

FFI has been found in just 40 families worldwide, affecting about 100 people. Small wonder it is currently incurable. May factors affect whether or not cures are found for diseases, such as research funds, availability of academic and professional resources, interested researchers, willing volunteers,  It takes significant money to conduct research on medical conditions and if only one parent has the gene, the offspring have a 50% risk of inheriting it. Personally, I don’t care for those odds. Onset is around middle age. One positive about the disease is that it is possible to test for it in order to avoid having offspring with the disease.

The disease progresses in four scary stages:

  1. The person has increasing insomnia, resulting in panic attacks, paranoia and phobia. This lasts for approximately four months.
  2. Hallucinations and panic attacks continue for about five months.
  3. Complete inability to sleep is followed by rapid loss of weight. This lasts for about three months. I’m not surprised by the connection to weight loss. In my blog 40 years of faulty wiring, I wrote about bizarre human diets, one of them being the Sleeping Beauty Diet whereby people use – ahem – pharmaceutical means to sleep for unnaturally long periods of time in an effort to lose weight.
  4. Dementia is the final stage where the patient becomes unresponsive or mute for six months. This is the final progression of the disease, after which death follows. I should think the reason for becoming mute is the psychotic break from reality caused by the insomnia, not to mention the brain’s inability to fully function any longer without sleep. If you want to read a brief paragraph about a man whose body was able to function physically after he had been axed to “death” read  Perturbing, Puzzling Parricide Puts Pretty Porco into Prison from my blog Killers Without Conscience. It’s fascinating what a brain can do.

The first modern case was reported in the Netherlands of a 57-year-old man of Egyptian descent. The man suffered  symptoms of double vision and progressive memory loss, he was disoriented, paranoid and confused. He experienced vivid dreams and random muscular jerks during normal slow wave sleep. The patient died at 58 (seven months after the onset of symptoms). In late 1983, neurologist/sleep expert Dr. Ignazio Roiter received a patient at the University of Bologna Hospital’s sleep institute. The man, known only as Silvano, decided in a rare moment of consciousness to be recorded for future studies and to donate his brain for research. The first case recorded on medical books is that of Giacomo, a man from Venice, Italy. Click here for his story.

Creepy Case Study
maxresdefaultOne of the most notable cases is that of Michael (Michel A.) Corke, a music teacher from New Lenox, Illinois. He began to have trouble sleeping before his 40th birthday in 1991; following these first signs of insomnia, his health and state of mind deteriorated. Eventually, sleep became impossible, and he was admitted to University of Chicago Hospital, with a misdiagnosis of clinical depression due to multiple sclerosis. Medical professionals. In a bid to provide temporary relief in the later stages of the disease, physicians tried to induce a coma with the use of sedatives, however  his brain failed to shut down completely. Corke died in 1993, a month after his 42nd birthday, by which time he had been completely sleep-deprived for six months. No, this is not a picture of Corke. It’s a gag that channels my blog about the RSI. Poor Corke.

Why We Sleep
Have you ever noticed how grumpy people are when they don’t get enough sleep? This is much deeper than simply being “grouchy.” Even partial insomnia leaves the brain unable to function at full capacity. Sleep deprivation makes people moody because they aren’t able to intellectually and emotionally relate to others as efficiently as normal.

Sleep is necessary for many reasons (aside from drowning out your nagging spouse) but these are the two biggies:

  1. repair damage to the brain from daily living, eg. excessive drinking
  2. rejuvenation of damage to the body and brain

Even one night of insomnia equals legal intoxication. It’s never wise to drive or to operate machinery. In fact, it’s always better to return to bed than to try to function as per usual. It won’t quite happen. Here is a creepy Law and Order SVU trailer about a girl who murdered her roommate partially as a result of extreme insomnia.







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