Batten disease, also known as neuronal ceroid lipfouscinosis (NCL), is a rare condition that affects children and causes the accumulation of lipofuscin in body tissues, leading to brain and organ dysfunction. Symptoms include vision problems, personality changes, and seizures. Treatments aim to address symptoms and provide comfort, with some promising developments in gene therapy and stem cell products. There is currently no known cure.
Batten disease is a rare condition that usually has its origins in childhood. The condition is known by several names. While Batten syndrome is another commonly used name for Batten disease, the condition is often referred to in medical journals and other academic work as Curschmann-Batten-Steinert syndrome or Spielmeyer-Vogt-Sjogren-Batten disease.
The common name for Batten’s disease is in honor of Dr. Frederick Batten, a British pediatrician who first took note of the condition in 1903. Batten’s research has led to the condition being identified and classified as a separate disease. His early research and breakthrough discoveries paved the way for other 20th-century researchers to discover more about the origins and function of the disease.
Although Batten disease is usually understood to be a form of neuronal ceroid lipfouscinosis (NCL) that affects children, it is not unusual for doctors and researchers to use the term as a collective way to identify each type of NCL. Essentially, the condition occurs due to the accumulation of lipofuscin in the body’s tissues. Over time these extra deposits of fat and protein in body tissues can interfere with the proper functioning of the brain and other key organs in the body. The result is a number of different health problems that can lead to death.
The symptoms of having Batten disease mimic many other health problems. Symptoms usually begin to appear between the ages of four and ten. Some of the most common signs of the possible presence of Batten disease are vision problems, a change in learning ability and attitude, personality changes, a decrease in motor activity leading to more frequent trips or falls. The child may also begin to experience seizures or episodes of feeling depersonalized.
Treatments for Batten disease usually involve trying 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 drugs derived from stem cell products also showed some promising results. However, at this time, there is no known cure for Batten disease.
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