April 14, 2005

In the news ...

Scientists at the University of NSW who have been working with border collie breeders to find the genetic cause of a rare inherited brain disorder, have announced that they have identified the genetic mutation responsible. Dogs affected with neuronal ceroid lipofuscinosis show increasingly severe physical and mental symptoms as their brains degrade and they rarely survive beyond two to three years old. The physical symptoms differ from breed to breed, Bulldogs first show gait problems, the first sign in Miniature Shnauzers is visual problems.

The breakthrough means a test will soon be available to screen for the disease in border collies and stop if from being passed on. DNA tests have previously been available for English Setters and American Bulldogs.

Known as Batten Disease, neuronal ceroid lipofuscinoses is thought to be one of the most common neurodegenerative diseases in humans, but it remains an unsolved mystery today, a puzzling disease that assures its victims of only one consistent manifestation...early death.
Batten may affect persons of any age, but primarily affects infants, toddlers and school age children, beginning unexpectedly and leading to a progressive loss of brain function that later destroys bodily functions, eventually leaving the victim totally helpless.

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