Monday, May 18, 2009

Lesch-Nyhan Syndrome

I'm a big fan of the Best American Writing series. If you're not familiar, the best America Writing series includes a number of yearly publications allocated to subjects like Travel or Science. In the 2008 Science series, there is an article on Lesch-Nyhan syndrome. Lesch-Nyhan syndrome is "a rare disease which causes people to mutilate themselves."

People with Lesch-Nyhan try to eat off their fingers and lips. They're often successful. They also do things like drive into traffic with their wheel chairs - in an attempt to harm their body. Lesch-Nyhan patients don't want to mutilate themselves, but because of a brain issue, their body does lugubrious things. It's an incredible disturbing syndrome that has no cure.

As I read about sicknesses like Lesch-Nyhan, I'm better able to put life into perspective. Making money, having a big house or driving a new car are all insignificant. Having a healthy family is the most important thing in the world.


Scooter said...

Rent or Netflix a copy of Whole by Melody Gilbert, a local documentary maker. I don't believe it's the same syndrome, but if you found the article interesting, you'll probably find her documentary interesting.

bosshart said...

If you have time, the New Yorker has had a couple articles in the past year that concern similar neurological syndromes or disorders - people itching through their scalp into their brain tissue, the desire to cut off their limbs, phantom limb pain, etc. They discuss some of the neurological origins and how they have been able to cure these disorders.

The first article discusses the itching and how they found out how to cure "phantom limb" pain in amputees using a mirror. It's pretty amazing, because you can cut the physical connection between the itch or amputated limb and brain and pain (or itch) will persist. But for amputees, this mirror treatment has something like a 90% success rate.

The most recent article is even better - unfortunately you have to register, but it talks about the "plasticity" of the brain - how the brain works. It makes connections between these neurological conditions to things like creativity, and other seemingly unrelated disorders like schizophrenia and autism. Of course, it's all just theory, but fascinating nonetheless.

Anonymous said...

It's good for us once in a while to get slapped in the face with the fact that we are incredibly blessed. When I worked downtown St Paul, I'd occasionally see a fellow who had no legs and one arm. He got around on a skateboard. I'd see him generally just after I'd complained about a hangnail. Slap!


Mac Noland said...

"Whole" looks far too disturbing for me.

Bosshart, I'll have to check out those articles. The New Yorker must have an interest in odd disorders.