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.

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.