Researchers Find Clues to Batten, a Deadly Childhood Disease

Researchers announced that they have found a new drug therapy that may prove to help children who suffer from Batten disease. In a recent press release, researchers from the University of Rochester Medical Center released details of their findings.

Batten disease is a rare disease that is a nuerodegenerative disease. It strikes without warning, usually attacking children who had been healthy before they are diagnosed with this disease.

Currently there is no treatment for this disease. None. Nothing stops or even slows its progression. Now researchers are on the cusp of finding a drug that holds the potential to help children suffering from Batten disease.

"Since deterioration of motor skills is the rule - in fact, it's one of the primary symptoms in children with the disease - the idea that these functions might be able to be partially restored or improved is groundbreaking," David Pearce, Ph.D., a biochemist at the University of Rochester. Pearce is also nationally renowned expert in Batten disease, having participated in over 50 different research studies about Batten disease.

While incredible amounts of research are still needed, this information provides at least a glimmer of light for the children and families affected by Batten disease.

Researchers discovered that the brain cells of mice that had Batten disease were particularly sensitive to glutamate. Glutamate is a neurotransmitter that is critical for the memory and learning process to function.

In identifying this neurotransmitter and region of the brain that houses it, researchers probed further to see what other features they could discover. They found that a drug was able to block some of these receptors, thus slowing or diminishing their activity.

In a nutshell, the mice that received a specific drug actually became more coordinated than they had been. While they never regained as much coordination as mice that were healthy, simply making any type of recovery whatsoever was remarkable by itself.

Batten disease is also known as Spielmeyer-Vogt-Sjogren-Batten disease. It is inherited, and strikes the nervous system of the children who develop it. As this disease progresses, children lose their ability to see, move, and think. It typically claims their life while they are in their early twenties.

Children who are affected by this disease typically show symptoms somewhere between the ages of 4 to 10 years old.

Researchers hope that this study will show promise in treating children with Batten disease and many other related diseases as well. Other diseases that are related to Batten disease include Krabbe disease, metchromatic leukodystrophy and Tay-Sachs.

Sources:
http://www.eurekalert.org/pub_releases/2007-12/uorm-run121207.php
http://en.wikipedia.org/wiki/Batten_disease