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What is Batten Disease?

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  • Written By: Malcolm Tatum
  • Edited By: Bronwyn Harris
  • Last Modified Date: 18 September 2016
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Batten Disease is a rare condition that usually has its origins in childhood. The condition is known by several different names. While Batten’s Syndrome is another commonly employed name for Batten Disease, the condition is often referred to in medical journals and other scholarly works as either Curschmann-Batten-Steinert Syndrome or Spielmeyer-Vogt-Sjogren-Batten Disease.

The common name for Batten Disease is in honor of Dr. Frederick Batten, a British pediatrician who first made note of the condition in 1903. Batten’s research led to the identification and classification of the condition as a separate disease. His early groundbreaking research and findings paved the way for other 20th century researchers to discover more about the origins and function of the disease.

While Batten Disease is normally understood to be a form of neuronal ceroid lipfouscinosis (NCL) that affects children, is it not unusual for doctors and researchers to use the term as a collective way of identifying every type of NCL. Essentially, the condition comes about due to the accumulation of lipofuscins in the tissues of the body. Over time these extra deposits of fat and protein in the body’s tissues can interfere with the proper function of the brain and other key organs in the body. The result is a number of different health issues that can lead to death.

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Symptoms of the presence of Batten’s Disease mimic many other health issues. The symptoms usually begin to appear between the ages of four and ten. Some of the more common signs of the possible presence of Batten’s Disease are problems with vision, a change in learning ability and aptitude, personality changes, an a decrease in motor activity that leads to more frequent stumbling or falling. The child may also begin to experience seizures or episodes of feeling depersonalized.

Treatments for Batten Disease usually involve attempting to address the symptoms and provide as much comfort as possible for the patient. In recent years, the use of gene therapy has led to some promising developments in treatment. Experiments conducted in 2006 using medication derived from stem cell products also have shown some promising results. However, at this time, there is no known cure for Batten Disease.

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