Topic: This disease freaks me out
cccuuunnttt !nIGga.SL0w started this discussion 15 years ago #11,948
http://en.wikipedia.org/wiki/Fatal_familial_insomnia
It's a condition, related to mad cow, that makes people totally unable to sleep, leading them to waste away and die. There are a number of families, perhaps 40-200 worldwide, who apparently have the inherited mutation. Sometime around the age of 40-50, they become totally unable to sleep, to regulate their sweating or pupil dilation, and within a year or two, they die.
Interview with the author of a book about an Italian family which has had the disease for generations, but only recently diagnosed:
http://www.npr.org/templates/story/story.php?storyId=6503414
It's a condition, related to mad cow, that makes people totally unable to sleep, leading them to waste away and die. There are a number of families, perhaps 40-200 worldwide, who apparently have the inherited mutation. Sometime around the age of 40-50, they become totally unable to sleep, to regulate their sweating or pupil dilation, and within a year or two, they die.
Interview with the author of a book about an Italian family which has had the disease for generations, but only recently diagnosed:
Silvano one day, in the Italian style, was dancing with his mother. And in Italy, you know, young bachelors are always dancing with their mothers, even today. And so he noticed he was sweating, which he knew from his experience with sisters was the first sign of the disease. And so Silvano understood what was happening. He'd always really known, I think, that he was going to get the disease. And then he went and looked in the mirror and he saw that his pupils had become these tiny pinpricks, and he knew for sure what was happening.
http://www.npr.org/templates/story/story.php?storyId=6503414
Anonymous B joined in and replied with this 15 years ago, 3 minutes later[^] [v] #214,927
> dominant inherited
OH GOD NO
OH GOD NO
Born this Way !dLadyGAgAA joined in and replied with this 15 years ago, 3 minutes later, 7 minutes after the original post[^] [v] #214,928
How do they live for 2 years without sleeping?
Anonymous D joined in and replied with this 15 years ago, 4 minutes later, 11 minutes after the original post[^] [v] #214,930
sluggy joined in and replied with this 15 years ago, 3 minutes later, 15 minutes after the original post[^] [v] #214,932
WHY ARE THEY REPRODUCING?! Unless it's all in the family. Then it's ok. I guess 40-50 years is enough to have a good life.
sluggy double-posted this 15 years ago, 1 minute later, 16 minutes after the original post[^] [v] #214,935
I get mad cow specimens at work, and there have been a lot more recently (suspected cases. 2 were positive).
Theres no cure. We have to take super precautions because it is so contagious.
They had CSF floating around the lab one day before we knew the patient had mad cow. Scary shit.
Theres no cure. We have to take super precautions because it is so contagious.
They had CSF floating around the lab one day before we knew the patient had mad cow. Scary shit.
brie !OudrPWaUDQ joined in and replied with this 15 years ago, 2 minutes later, 19 minutes after the original post[^] [v] #214,939
> The age of onset is variable, ranging from 35 to 60, with an average of 50. However the disease tends to prominently occur in later years, primarily following childbirth.
I've heard of this before, but always wondered how exactly the disease managed to be 'familial'. I guess this is how.
It'd be interesting if this became a successful mutation and we evolve into beings that just die at 50. Over-population is a bitch, this could be a nice genetic solution to the whole thing..
I've heard of this before, but always wondered how exactly the disease managed to be 'familial'. I guess this is how.
It'd be interesting if this became a successful mutation and we evolve into beings that just die at 50. Over-population is a bitch, this could be a nice genetic solution to the whole thing..
cccuuunnttt !nIGga.SL0w (OP) replied with this 15 years ago, 3 minutes later, 22 minutes after the original post[^] [v] #214,940
@214,932 (sluggy)
It's hard to understand. I read part of the book mentioned above, and it sounded like for the Italian family at least, it was just sort of rumored to be odd mental illness and a bunch of random other explanations. They only found out very recently what they were dealing with.
It's hard to understand. I read part of the book mentioned above, and it sounded like for the Italian family at least, it was just sort of rumored to be odd mental illness and a bunch of random other explanations. They only found out very recently what they were dealing with.
Anonymous G joined in and replied with this 15 years ago, 3 minutes later, 26 minutes after the original post[^] [v] #214,941
@214,930 (D)
God, I hate those. I always wake up thinking someone saw what I was doing on the computer until I look at the clock.
God, I hate those. I always wake up thinking someone saw what I was doing on the computer until I look at the clock.
cccuuunnttt !nIGga.SL0w (OP) replied with this 15 years ago, 14 minutes later, 41 minutes after the original post[^] [v] #214,945
@214,939 (brie !OudrPWaUDQ)
There's a theory that essentially everyone else has a trait to protect them from prions (as a result of practicing cannibalism perhaps). These families merely lost that at some point. I'm not sure how it could be a benefit, wouldn't people with the disorder reproduce less at some point when it's fully figured out, so basically it would have to keep randomly happening all the time?
@previous (G)
wat
There's a theory that essentially everyone else has a trait to protect them from prions (as a result of practicing cannibalism perhaps). These families merely lost that at some point. I'm not sure how it could be a benefit, wouldn't people with the disorder reproduce less at some point when it's fully figured out, so basically it would have to keep randomly happening all the time?
@previous (G)
wat
sluggy replied with this 15 years ago, 5 minutes later, 46 minutes after the original post[^] [v] #214,948
@previous (cccuuunnttt !nIGga.SL0w)
Its late onset is probably why. Evolution favors quick reproduction over longevity.
If it's onset is after people are likely to have reproduced, and there is no signs of the illness when younger, there's no real mechanism to limit it.
Its late onset is probably why. Evolution favors quick reproduction over longevity.
If it's onset is after people are likely to have reproduced, and there is no signs of the illness when younger, there's no real mechanism to limit it.
Anonymous G replied with this 15 years ago, 9 hours later, 9 hours after the original post[^] [v] #215,063
@214,945 (cccuuunnttt !nIGga.SL0w)
I sometimes fall asleep at the computer for short periods of time and wake up in fits of paranoia that someone might have seen my history or folders until I see that it hasn't been long enough for that to happen.
I sometimes fall asleep at the computer for short periods of time and wake up in fits of paranoia that someone might have seen my history or folders until I see that it hasn't been long enough for that to happen.
Ks !KansasxqvM joined in and replied with this 15 years ago, 3 hours later, 13 hours after the original post[^] [v] #215,102
Fuck. That. Shit. I've had insomnia off and on for years, I imagine dying from this disease is some kind of long, drawn-out, horrible, waking nightmare...
swamplights !!V3J448+/B joined in and replied with this 15 years ago, 1 hour later, 14 hours after the original post[^] [v] #215,120
We once had a patient with Creutzfelt Jakob disease which is the human version of mad cow disease. They investigated everywhere and never figured out how he caught it.
http://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
http://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
Ks !KansasxqvM replied with this 15 years ago, 22 minutes later, 14 hours after the original post[^] [v] #215,121
Anonymous J joined in and replied with this 15 years ago, 13 minutes later, 15 hours after the original post[^] [v] #215,125
On the topic, kuru is another transmissible spongiform encephalopathy caused by a prions and related to Creutzfelt Jakob disease. It is characterized by tremors leading to a total loss of muscle control and sudden bouts of laughter during onset -- no mention of sleep disorders though.
swamplights !!V3J448+/B replied with this 15 years ago, 18 minutes later, 15 hours after the original post[^] [v] #215,146
@215,121 (Ks !KansasxqvM)
The man was always awake and shaking. At the beginning of his stay where I work, he could walk and move, with only a slight tremor. He was also lucid and coherent, like a normal dude with a slight twitch. As the disease progressed he began shaking a little harder and began "losing words". His motor skills started getting bad and he would try to pick things up and knock them over. Or he would pass his hand several feet in front of the object he would reach for.
The man was always awake and shaking. At the beginning of his stay where I work, he could walk and move, with only a slight tremor. He was also lucid and coherent, like a normal dude with a slight twitch. As the disease progressed he began shaking a little harder and began "losing words". His motor skills started getting bad and he would try to pick things up and knock them over. Or he would pass his hand several feet in front of the object he would reach for.
