Fatal Familial Insomnia

Fatal Familial Insomnia is one of the most interesting diseases known to mankind. It is a state of sleeplessness that begins during middle age; once your child-bearing years are over. FFI is very rare, affecting less than 40 families world-wide at time of posting. This ticking time bomb is caused by a rare genetic mutation whose nature links this it to the likes of Kuru, Mad Cow Disease, and Alzheimer’s Disease.

Fatal Familial Insomnia is known as a Prion disease (as are those mentioned above). A Prion is a name given to an infectious agent and in the case of FFI it is caused by a mis-folded protein that permanently affects the structure of the brain.

The prions eat away at the Thalamus region, which is the part of the brain responsible for the regulation of sleep as well as other sensory and motor functions. Due to the disease not presenting until middle age, the disease has been unknowingly passed down through whole familes. You can read about the fascinating case of one poor family in the book The Family That Couldn’t Sleep.

The documenary Dying To Sleep can be seen here:

There is now a genetic test, but unforunately no known cure as yet.

This deadly brain disease manifests itself in four stages :

1) The inexplicable and sudden onset of insomnia can cause unfounded phobias and panic attacks. This stages last for about 4 months.

2) When sleep deprivation takes hold, the hallucinations and panic attacks may become severe. This stage lasts for about 5 months.

3) Insomnia starts causing weight loss, and restricts proper mental functioning. The duration of this stage is about 3 months.

4) Finally, the person suffers unresponsiveness and dementia. This stages lasts for about 6 months.

Eventually, the person suffering from fatal insomnia falls into a state of coma, and dies. One of the most unfortunate aspects of this disease perhaps, is that the person has a clear understanding of the condition, while experiencing the physical agony induced by total sleeplessness.