Going Postal 1

As it is the week of my blogiversary, I decided the laziest best way to celebrate would be to have some reposts. Today we’ll have the post with the most page views, according to my stats page. And the winner is…

There is nothing to fear but fear itself. Also spiders. [originally posted Monday, December 20, 2010]

I read an interesting article the other day. It seems this woman (only identified as SM) isn’t afraid of anything. I mean that when she’s exposed to things that normally scare people (spiders, threats) she barely feels anything. She has been in violent situations, scary situations (domestic violence, held up at knifepoint), but still isn’t afraid. The question is why.

Part 1: The Disease

SM fascinates the doctors studying her. Due to Urbach-Wiethe, an extremely rare genetic disorder (there are only three hundred known cases in medical literature), her amygdala is damaged. The disease causes physical symptoms like thickening of the skin and mucous membranes, and can cause epilepsy, attacks of rage and mental retardation. There are other neurological symptoms, too, including schizophrenia and mood disorders. I think it’s safe to say that the neurological phenomena occur based on where the UW affects the brain.

Urbach-Weithe disease is caused by (according to this article) “a defect in the metabolism of basement membrane collagen.” For everyone who isn’t a doctor, the basement membrane according to Medterms.com is “A thin membrane upon which is posed a single layer of cells…made up of proteins held together by type IV collagen.” It’s located directly under the epidermis, hence the reason it’s called “basement.” In UW disease, the basement membrane doesn’t regenerate (metabolize) properly, resulting in lipids (fats) being created throughout the basement membrane. This results in a lot of problems in the skin, mucous membranes, eyes and even the brain. The disease is usually detected in children because of reports that their voice is hoarse and cuts and scrapes don’t heal properly. The disease causes both; the hoarseness is caused by the calcification of the vocal cords among other things, while the scars are caused by improper healing.

The calcification builds up and affects other parts of the body: the eyes, scalp, and of course, the brain.

So now we know how the brain is altered. Tune in tomorrow as we continue to probe the mystery of the fearless woman.

I don't think I did a very good job writing the article (forgive me! It was only my third month in my blog) but it was still an interesting subject. If you check the old post, you'll see that there are only four comments, (I love that two of them are from William and Kathy, who I'm still blogging buddies with). But this is indeed my most viewed page. If you just have to see the rest of what I wrote about fear, here are links to parts two and three.

Fearless

I am not actually without fear. I believe spiders will try to crawl into my mouth if I’m not careful because they are unholy creatures even Satan shuns. I’m also not a fan of public speaking unless, like here, I can craft my words via typing. But when it comes to movies, books and video games, I’m rarely frightened. This isn’t a shock since I literally grew up reading Stephen King (I was the only fifth grader who brought CHRISTINE to class to read) and was similarly indoctrinated to horror movies (I remember watching Alienwhen I couldn’t have been more than seven). So, in summation, it takes a lot for a book/movie/game to scare me.

There’s a reason I threw “game” in there. While roving through the internet one day when I should have been editing, I came across a story about “the Slender Man”. It’s an urban legend actually invented on the internet, about a tall, thin man in a black suit with no face, just white where eyes and a mouth should be. In all a good story. Nothing to write home about, but hey. It’s different from the usual ghosts and ghouls.

Except. There is a game called Slender, released free for download over the internet. I heard about it and decided to check it out on YouTube and…it was scary. Actually scary. Silent Hill was interesting, but didn’t make me blink and this, released as an indie game, actually frightened me enough that I didn’t want to download it, the crummy YouTube videos were enough for me. Not long after I stumbled across another game, Deep Sleep. It’s a Flixel game and so the graphics aren’t on par with Slender, but it’s still creepy as sh!t. It’s also a short point-and-click game, where you collect items to use to escape a typical scary setting. For such a quick game, it sure gave me chills.

What these games have in common is the lurking figure, the one that remains just out of sight and hunting you. Neither has a strong story or any characters except the first-person player, but those aren’t necessary. You are the character, something is after you, and you have to keep moving. A simple concept, but don’t go mistaking that for a criticism. If you want a good story, look elsewhere. If you want to be scared, go for either one of these games.

And leave the lights on.

Scarefest

I have a confession to make: while I enjoy scary movies and books, for the most part, I’m not scared by them. Okay, it’s kind of a strange confession, but it’s true. I might jump if I’m in a theater (because anything is scary in a movie theater), but that’s it. There isn’t much terror in it. If it’s not the first time I’ve watched it, forget about it. The same goes for books, too. Maybe this is because I’ve read Stephen King ever since I was eight, but I don’t ever remember being sleep-with-the-light’s-on-or-the-monster-will-get-me scared.

Am I weird? Eh, whatever. It doesn’t stop me from enjoying it. Unless it’s a bad movie. Because those just ruin everything. But if you’re looking for something scary, you might want to try the books and movies that actually scared me:

The Orphanage

Fright Rating: 2 (Out of 5)

This was a Spanish movie that received a limited release in the US. I had to travel across the state to see it (not as far as it sounds, trust me) but it was worth it. While it wasn’t super scary, it gave me chills. It was an emotionally driven movie about a family who bought a house that twenty years ago, was the orphanage the mother lived in. Ghostly things start happening and the end was quite a punch to the gut. If you don’t mind reading subtitles, try to pick up a copy.

IT

Fright Rating: 2

Stephen King’s tale of the monster that wakens every twenty-seven years or so to stalk children of a town in Maine. Parts of this book still give me tingles and I’ve probably read it a dozen times. I think the scariest part is Mike Hanlon’s reflections about the nature of the word haunt (is anyone surprised that word play drew my attention?) and what it means to the town of Derry.

The Exorcist

Fright Rating: 3

I’m so glad I was able to see the rerelease in the theater. It really creeped me out, even though it wasn’t the first time I saw the movie. The basic plot is about a girl, daughter to a movie star, who is seemingly possessed. The book delves deeper into the “is she faking it?” question and is at least as scary as the movie, perhaps more so because there’s more time for the tension to build and more tension always encourages horror.

Paranormal Activity

Fright Rating: 3

Found footage movies are kind of this generation’s slasher movies. Some people hate them, but most of them scare me (unlike slasher movies, which I find to be yawn-fests). I thought Paranormal Activity was well done because a lot of the fear hinges on how real it seems. And it’s not gory or full of monsters. It’s just two people trying to make sense of evidence that neither is prepared to deal with.

The Statement of Randolph Carter

Fright Rating: 3.5

Actually, this goes for pretty much everything of Lovecraft’s that I’ve read, including Herbert West—Reanimator, The Call of Cthulhu, and the Whisperer in Darkness. All scared me a pretty decent amount. I think that is due to Lovecraft’s skill at building tension and describing people who are so frightened, that they would rather jump out of a window rather than face the demon (in all fairness, it’s a pretty bad demon).

House of Leaves

Fright Factor: 3.5

This is a great example of realistic horror. No monsters (okay, that’s arguable), just a situation that is so inexplicable that it’s frightening. It’s also very character driven, which I love. Most of the fear comes from this family who returns from a short vacation to find their house has gained new hallways, one of which should lead out into the back yard but instead goes into a cold black corridor.

So these are my recommendations for the Halloween season. Enjoy the scarefest! And all hail great Cthulhu!

Spiders 3: The Terrifying Conclusion!

So, did you like this diversion into the realm of physiology? I sure did. If you want to write emotion, you have to understand it. And now I know more about fear.

The human mind, when you really take the time to think about it, is an amazing thing. It evolved, from a few cells whose purpose was to use past experiences to anticipate future problems to an organ capable of controlling vital functions without its creating even being aware of it. One malfunctioning piece, though increasing vulnerability, does not necessarily bring about its end.

In fact, we humans can alter our brains if we want. We can learn new information to use, we can communicate with others, we can teach ourselves ways around the mental road blocks we are all built with. From our brains we have created beauty, learned about the quantum foam of the universe, carved out cities that outlast their inhabitants by centuries. All this from a few cells, from a unicelled organism, from some amino acids, from random proteins, from primordial ooze, from high energy, self-replicating molecules, from molecular gas collapsing into a star, from gravity constantly pulling things and making them interact with each other.

Amazing.

Sources

Speigel, Lee. “Scientists Study Woman With No Fears.”

Dec 19, 2010. http://www.aolnews.com/2010/12/19/scientists-study-woman-with-no-fears/?icid=main|aim|dl1|sec3_lnk1|190886

Batra, Kadambari; Safaya, Anil; Aggarwal, Kiran. “Lipoid proteinosis (Urbach-Wiethe disease): a case report from India.” Ear, Nose and Throat Journal. Sep 1, 2008. http://www.thefreelibrary.com/Lipoid+proteinosis+%28Urbach-Wiethe+disease%29%3A+a+case+report+from+India.-a0186320446

Medterms.com definition: Basement membrane. http://www.medterms.com/script/main/art.asp?articlekey=39157

“Lipoid Proteinosis (Urbach-Wiethe Disease).” [http://staff.aub.edu.lb/~webderm/downloads/cases/15.pdf]

Siebert, Michaela; Markowitsch, Hans J.; Bartel, Peter. “Amygdala, affect and cognition: evidence from 10 patients with Urbach–Wiethe disease.” Brain. August 22, 2003. http://brain.oxfordjournals.org/content/126/12/2627.abstract

Samiullah, Javeria; Neelofar, Marium; Samad, Fatima; Nabi, Ghulam; Ghazal, Saadia.

 “Urbach-Wiethe Disease: Experience at a Tertiary Care Hospital in Abbottabad, Pakistan.” J Ayub Med Coll Abbottabad. 2008. http://www.ayubmed.edu.pk/JAMC/PAST/20-4/Javeria.pdf

Prasad, P. V. S.; Sahoo, G.C. “Lipoid Proteinosis (Urbach Wiethe Disease)—A Case Report.” Indian Journal of Otolaryngology and Head & Neck Surgery. Volume 52, Number 2, 172-173. http://www.springerlink.com/content/lht6u307267q2409/

Part 2

Black, Harvey. “Amygdala's Inner Workings.” The Scientist. Volume 15, Number 19, 20. October. 1, 2001.

H. Kluver, P.C. Bucy, "Preliminary analysis of the temporal lobes in monkeys," Archives of Neurological Psychiatry, 42:979-1000, 1939.

Spiders 2: The Physiology of Fear

Anxious? Then, most likely, the amygdala is to blame. People with damaged amygdalas (like SM mentioned yesterday) have abnormal emotional responses. SM doesn’t feel fear, and scans of her brain show an amygdala full of lesions. Scientists (H. Kluver and P.C. Bucy in the thirties and N.H. Kalin in 2001) damaged the amygdalas in monkeys (poor things) and noted that they became completely tame—they lost their fear responses.

The amygdala is the part of the brain that remembers when something bad happens and tells us how to react. Anxiety, fear, and aggression are all controlled by it, meaning a malfunctioning one can create anxiety disorders, depression, phobias, and even autism and schizophrenia. That isn’t to say it’s all physiological; post-traumatic stress disorder is also related the amygdala, but it is an acquired condition, not a preexisting one. And since cognitive behavioral therapy also helps with the above disorders, a “broken” amygdala can sometimes be “fixed.”

Psycheducation.org has some nice pictures of the amygdala’s location. If you go back to the fear article about SM, you can see that the pictures of her brain are missing an amygdala. Growing up with no fear (or anger, if Wisegeek is correct), she can’t discern the emotion on anyone or associate danger with situations. She might remember being attacked, but she has no instinctual response, i.e. if someone grabs her, she won’t reflexively fight back. There is no “fight or flight” with her. Just “huh; this is happening.”

While not knowing fear or anxiety might sound like a good thing, in reality, it’s pretty dangerous. SM walks down a dark street and fear doesn’t make her tighten her hand on her purse or listen for following footsteps. If a bomb goes off, she won’t duck unless she actually thinks “Oh, I should duck before a piece of shrapnel hits me.” And the amount of time it takes her to think that might take one second longer than she has before a shard of metal embeds itself in her skull.

Tune in tomorrow for concluding thoughts and credits. I know. I'm really drawing this out, aren't I? I'll post some cute cat pictures on Thursday. Hey. It'll be my hundredth post. I can do what I want. Peace!

There is nothing to fear but fear itself. Also spiders.

I read an interesting article the other day. It seems this woman (only identified as SM) isn’t afraid of anything. When she’s exposed to things that normally scare people (spiders, threats) she barely feels anything. She has been in violent situations, scary situations (domestic violence, held up at knifepoint), but still isn’t afraid. Doctors suspect the reason is lesions her amygdala. And I want to know more.

Part 1: The Disease

Urbach-Wiethe disease causes physical symptoms like thickening of the skin and mucous membranes, and can cause epilepsy, attacks of rage and mental retardation. There are other neurological symptoms, too, including schizophrenia and mood disorders. So how does this happen?

U-W is caused by (according to this article) “a defect in the metabolism of basement membrane collagen.” For everyone who isn’t a doctor, the basement membrane according to Medterms.com is “A thin membrane upon which is posed a single layer of cells…made up of proteins held together by type IV collagen.” It’s located directly under the epidermis, hence the reason it’s called “basement.” In UW disease, the basement membrane doesn’t regenerate (metabolize) properly, resulting in lipids (fats) being created throughout the basement membrane. This results in a lot of problems in the skin, mucous membranes, eyes and even the brain. The disease is usually detected in children because of reports that their voice is hoarse and cuts and scrapes don’t heal properly. The disease causes both; the hoarseness is caused by the calcification of the vocal cords among other things, while the scars are caused by improper healing. Check here for more.

The calcification builds up and affects other parts of the body: the eyes, scalp, and of course, the brain.

So now we know how the brain is altered. Tune in tomorrow as we take a closer look at the amygdala, the fear center of the brain.