BRAIN DEVELOPMENT IN DOWN SYNDROME : UPDATES ALSO LOOKING AT THE CAUSE OF MICROENCEPHALY – SMALL BRAIN.

Recent research may be the forerunner to a range of new treatments for those with small brains aka microencephaly. Typically, in Down Syndrome, the brain development in-utero is where most / all of the damage occurs.

C. N. Svendsen et al, 2002

Andrew Chess et al, 2004

Professor Roger H. Reeves, 2006

2002, C. N. Svendsen et al
STEM CELL RESEARCH AND BRAIN DEVELOPMENT IN DOWN SYNDROME

Down syndrome affects nearly 350,000 people in the United States.

Basis of study: Identifying the genetic problem that gives rise to Down Syndrome may lead to better treatments – possibly gene therapies and new drugs.

The research found that in Down Syndrome stem cells, there was a definite lack of sufficient cells that go on to form neurons, that the neurons that did develop were shorter and misshapen, when compared to non-Down syndrome stem cells.

In other words, contrary to popular thinking, the research has apparently demonstrated that babies, kids with Down Syndrome, have already suffered much brain deformation, mental retardation and IQ loss before they are born. This, in turn, puts to rest, puts to death, the idea that tni ( targeted nutrient intervention aka vitamin therapy ) administered to a baby or kid will prevent the brain being damaged – as the brains of the kids with Down Syndrome are clearly suffering much of the damage in-utero.

2004, Andrew Chess et al
The brain has over one trillion cells, with each cell now appearing to have it’s own unique identity.

The Dscam protein appears to be responsible for the ‘name’ of each cell. The Dscam protein may be present in several different forms in each cell, thereby giving the cell its ‘name’, this then allows other brain cells to ‘talk’ to that cell in a meaningful way. In Down syndrome, however, the extra chromosome also provides an extra Dscam gene as well. The extra Dscam protein that is conceivably produced as a result, might mess up the name of the cell and hence interconnections between the brain cell and other brain cells. It would be like a person called Robert in a crowded room, someone calls out “Rodger”, but Robert doesn’t know they mean him, so Robert ignores the yell. This lack of communication between brain cells may be a significant part of the cause of the learning difficulties common in those with Down Syndrome.

2006, Professor Roger H. Reeves
Prior to Prof. Reeves study, it was thought the brain cell death rate was much higher in those with Down Syndrome and that it was the higher cell death rate that caused the brain not to grow.

Working with Down Syndrome mice, Prof Reeves has shown that the cerebellam ( brain part responsible for motor skills and balance ) fails to grow properly because of a lower mitosis rate ( lower cell reproduction rate ). Further, he showed that by proper drug administration, he and his other researchers could increase the mitosis rate in the cerebellam of Down Syndrome baby mice, which resulted in the mice having a normal size cerebellam.

It is thought that this may be the precursor to new drug treatments for Down Syndrome babies. The researchers also postulate that similiar lack of growth may occur in the hippocampus – brain part responsible for memory and spacial orientation – and that this may be ameniable to drug treatment to stimulate growth while the baby is still in the uterus.

Lets hope that they will also find a way to stimulate the growth in those with Down Syndrome who are older than babies, even if it means an operation to open the skull up to allow for more growth.