Fatal Familial Insomnia

File this one away for your late-night hypochondria: it's got a lovely Fall of the House of Usher vibe to it.

The average survival time for patients diagnosed with FFI after the onset of symptoms is 18 months.

The disease has four stages:

  1. The person has increasing insomnia, resulting in panic attacks, paranoia, and phobias. This stage lasts for about four months.

  2. Hallucinations and panic attacks become noticeable, continuing for about five months.

  3. Complete inability to sleep is followed by rapid loss of weight. This lasts for about three months.

  4. Dementia, during which the patient becomes unresponsive or mute over the course of six months. This is the final progression of the disease, after which death follows.

Other symptoms include profuse sweating, pinpoint pupils, the sudden entrance into menopause for women and impotence for men, neck stiffness, and elevation of blood pressure and heart rate. Constipation is common as well. As the disease progresses, the patient will become stuck in a state of pre-sleep limbo, or hypnagogia, which is the state just before sleep in healthy individuals. During these stages, it is common for patients to repeatedly move their limbs as if dreaming.

The Tragic Fate Of The People Who Stop Sleeping:

Silvano eventually referred himself to the University of Bologna's sleep unit for further study, but he was under no illusions about the course of the disease. "He said, 'I'll stop sleeping, and within eight or nine months, I'll be dead,'" one of his doctors, Pietro Cortelli, told me in a phone interview.

"I said 'how can you be sure?' He then drew me his genealogical tree from the 18th Century, all by heart." In each generation, Silvano could name family members who had succumbed to the same fate. [...]

Whereas Creutzfeldt-Jakob disease leaves the surface of the brain looking like Swiss cheese, Silvano's condition seems to target parts of the thalamus, at the very centre of the skull. Normally the size and shape of a walnut, the thalamus in Silvano's brain appeared to have been riddled with boring worms. [...]

Daniel was determined to try as many potential treatments as possible, ranging from vitamin supplements and exercise to improve his general fitness to anaesthetics such as ketamine and nitrous oxide, and sleep medicines like diazepam -- anything that would give him a few winks of sleep, even for as short as 15 minutes at a time. He even bought a sensory deprivation tank, having found that even under an anaesthetic, his fragile slumbers could be interrupted by the slightest sound or movement.

Floating in the egg-shaped cocoon while bathed in warm salt water, he found the rest that had proven so elusive, enjoying a blissful four-and-a half hours of solid sleep. Once he awoke, however, he had to face terrifying hallucinations -- including a strange uncertainty as to whether he was alive or dead.

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One Response:

  1. Zippington T. Whatsis says:

    This seems related, although, everyone's probably already read it:

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