Live to sleep, sleep to live!

In response to Kyle’s post on a Vietnamese man who, apparently, hadn’t slept in 30 years, I ran a Google search on “sleep disorders” and (among other things) turned up some information on Fatal Familial Insomnia (FFI).

Here is what Wikipedia has to say about FFI:

Fatal familial insomnia (FFI) is a very rare, autosomal dominant inherited, disease of the brain. It is caused by a mutation in a protein called prion protein (PrP): asparagine-178 is replaced by aspartic acid. The mutation changes the shape of PrP so that it becomes a prion and makes other, normal PrP molecules change to the abnormal shape. This causes amyloid plaques in the thalamus, the region of the brain responsible for regulation of sleep patterns. The dysfunction of the thalamus first results in insomnia, which progresses to more serious problems over several years. The age of onset is variable ranging from 30 to 60. Death usually occurs within 3 years of onset. The presentation of the disease varies considerably from person to person, even among patients from within the same family. Common symptoms and signs include:

• intractable insomnia
• dysfunction of the autonomic system - causing hyperthermia, hypertension, tachycardia, tachypnea and hyperhydrosis
• dementia
• motor paralysis.

There are other "prion diseases" (TSEs) with different symptoms, such as Creutzfeldt-Jakob disease (CJD) and new variant CJD (vCJD) in humans, bovine spongiform encephalopathy (BSE) in cows, and chronic wasting disease in American deer and American elk (in some areas of the Rocky Mountains). FFI, as with other prion related diseases, is ultimately fatal and incurable. Hopes rest on the so far unsuccessful gene therapy and possibly drug development.

And here’s a link to more detailed information on the condition at the John Hopkins University “Online Mendelian Inheritance in Man” database:

http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=600072

Prolonged sleeplessness generally leads to various somatic and psychological disorders and even death. I suspect the man in the article cited by Kyle is engaging in “microsleep”, short bursts of sleep interspersed between long wakefulness periods. It is not really credible that the man has been truly “sleepless” continuously for 30 years.

Even “fragmented” sleep, i.e. periods of sleep interrupted at irregular intervals throughout the night can leave a person in good condition to perform physical work. I was a subject in sleep deprivation experiments while a graduate student at Indiana University and although fragmented sleep was annoying, my performance on the tread-mill tests the following day was comparable to my control performance after a normal nights sleep.