Battens
Battens Disease is a neurological disorder, which was discovered in 1903 by a British pediatrician. Battens Disease, also known as Spielmeyer-Vogt-Sjogre-Batten disease is the most common in a group of disorders called neuronal ceroid lipofuscinoses (NCLs). There are three other types of NCLs, which occur at different stages of life. The three other types of NCLs are infantile (6 months), late infantile (2-4 yrs.), and adult (before 40). A person inherits Battens when both parents give a recessive gene to the child. Battens is an autosomal recessive disease that is fatal. Battens Disease is a juvenile disease, which begins between the ages of 5-9 years, and it happens about once in every 25,000 live births. Since the disease has no outward signs the child appears normal at first. The individual develops just like a child without the disease does. Once the child reaches the age of 5-9 yrs. The symptoms start to develop. The first symptom that occurs is the deterioration of the child's vision. Other symptoms that start at the same time are problems with schoolwork, speech problems, and the onset of extreme clumsiness. After these symptoms have occurred the individual will start to have epileptic seizur
The atmosphere needs to be a loving one because the child can understand what is happening to them and around them. Currently the scientists are researching to find out what this protein has to do with NCLs. Therefore there are many different ways in which Battens Disease can be tested. Once the 10-15 years of the person's life are up the medical bills usually cost way over 1 million dollars. Then over time the lipopigments build up. When they are viewed under a microscope they appear a yellowish-green color. A person with Battens usually only lives to be it their late teens to early twenties. The home environment really is what ends up having to change. The parents and family around the child have to change their lifestyles. Both those types are used to get a picture of the brain while it is working. The family has to make sure that that the child with Battens gets the right nutrition. All these symptoms of Battens and the symptoms of the other NCLs have been linked to a buildup of substances called lipopigments in the body's tissues. Although it is known that these lipopigments build up in the cells of a person with Battens Disease, it isn't known what causes the buildup. So, when the parents of a Battens stricken child first find out, that their child has the disease, they view is as a death sentence.
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