Battens essays

Battens essays 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 childs 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 seizures. In the nest stage of the disease the individual will display gradual intellectual deterioration, and the gradual deterioration of muscles. The individual may even become demented over the coarse of the disease. All these things will happen over the coarse of 10-15 yrs. Near the end of those 10-15 years, which is also near the end of the individuals life, the person will spend much of his/her time lying down and they will be blind and unable to speak. All these symptoms of Battens and the symptoms of the other NCLs have been linked to a buildup of substances called lipopigments in the bodys tissues. Lipopigments are mainly composed of fats and proteins. When they are viewed under a...