Confined in the Playpen

2. November 2010

Some look at it as the key to stop ageing, others only as the rare case of a development disorder. A girl in the US is seventeen years old and still a baby. No gene-investigator was able yet to reveal the secret of her youth.

The world has not seen anything like this ever before. Brooke Greenberg might be the only case study for eternal youth among eight billion people. For many researchers, this American girl, by now 17 years old, is the incarnate ideal research object regarding “ageing processes”. The German newspaper Süddeutsche Zeitung quotes her physician Richard Walker at the University of South Florida/USA as follows: “Without trying to be demanding a sensation, this is an opportunity to answer the question why we are mortal.” But until today, the researchers did not manage to provide an answer why Brooke Greenberg’s body refuses to grow up.

Age: seventeen, weight: seven and a half kilograms

Brooke is 76 centimeters tall, weighs almost seven and a half kilograms and looks and acts like a child of about one. Over the past ten years, she gained about 1.5 kilograms and grew seven centimeters. Already in the womb, the fetus refused to grow continuously. Periods of normal development were followed by times showing significant delays. The pregnancy was finished in the 36th week with a premature birth. Brooke was born with 1.85 kilograms. The most obvious abnormity was an anterior hip dislocation.

Until today, Brooke walks with a trolley and is fed through a feeding tube. Esophagus and respiratory system have developed different and those muscles responsible for transportation of food from mouth via throat into stomach are working uncoordinatedly. Similar coordination disorders caused that the girls was not able to sit without help until she was six.

Brain tumor and stomach ulcers

But it’s not only the body that is affected. Also the mental development does progress but in extreme slow motion. She recognizes herself in the mirror and her closest attachment figures, reacts to simple orders like grabbing for things. But this is the typical behavior of an infant not able to talk yet.

What chances does Brooke have to get old and in doing so – continue developing further? If you take a look at the medical history of this seventeen-year-old, you will be surprised that the retarded teenager is still alive. By now, Greenberg has survived a stroke and a tumor with spontaneous remission. About twelve years ago, physicians diagnosed a tumor several centimeters big in her brain. Much to the surprise of all observers, the tumor vanished soon and all by itself.

Unexplained genetic defect

In spite of their intensive search, even experienced human geneticists were not able to find any really remarkable genetic defects in her genotype. Many experts consider the activity of the telomerase to be the key to ageing processes. It is very active in cancer cells and thus causes the immortality of the cell. At least in Brooke Greenberg’s skin-fibroblasts, the researchers did not find any telomerase activity. On the contrary: The average length of the telomeres on the chromosomes is less than those (according to the birth date) of young adults of the same age. No striking features also in the hormones responsible for growth and development. A treatment with somatotrophin did not change anything about the development disorder. Mr. and Mrs. Greenberg have more children: A younger and two older sisters, all healthy and developed normally.

Werner’s syndrome and Hutchinson-Gilford syndrome are different forms of progeria. Contrary to Brooke Greenberg’s phenotype, the body there ages ahead of schedule and very fast. A defect in the responsible genetic regions? Nil return.

Master-gene for coordinated organ development

In the meantime the experts rather agree that when investigating the background the defect of a gene is not causing slow or fast ageing. Because only certain parts of her body age very slowly, like for example most of her milk teeth are in good order. They match those of an eight year old, her bones have a biological age of about ten. The esophagus on the other hand is unnaturally small.

Since the tracing mostly failed so far, there is plenty of room for hypotheses. Richard Walker published the one seemingly making sense about a year and a half ago in ”Mechanisms of Ageing and Development“. He believes in a defect in a “Master-gene” responsible for coordinated growth of organs in a body. This gene is supposed to take care that in each development phase of a person the cooperation functions between organs with different growth speed. The problem there: Brooke Greenberg’s case is the first and only case of such a syndrome to be found in literature. There might be a second person in Brazil with the ageing stop. But until today no details appeared in any professional journals or magazines.

When you are looking at photographs of Brooke Greenberg at the age of five, ten eleven and fifteen, you will have a problem sorting them in the right order. Does this family hold the key for eternal youth? The price is high for Brooke’s Mom – she has to take care of an infant every day – and did so for the past seventeen years.

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