Batten is a fatal, inherited condition of the nervous system which typically begins from the childhood.
Batten is a fatal, inherited condition of the nervous system which typically begins from the childhood. The early signs may begin to appear between 5 to 10 years of age, when a normal child begins to develop vision problems or seizures. While these early signs may be subtle in some cases, taking the form of personality, behaviour changes, slow learning, clumsiness or stumbling. As the times passes, the affected children may develop mental illness, progress loss of sight, motor skills and the seizures may become worse. Affected children eventually become blind and demented. The condition may become fatal in late teens and twenties.
The symptoms of the condition are linked to a substance made of fats and proteins called lipofuscins( lipopigments) in the body tissues. The lipopigments build up in the brain cells, skin and eyes, muscles and many other tissues and are found inside a part of the cells called lysosomes. These lysosomes are responsible for getting rid of the damaged things or which are no longer required. The accumulation of lipopigments in Batten disease can be seen under an electron microscope which looks like half moons or fingerprints. Genes which are linked to condition are PPT1, TPP1, CLN3, CLN5, CLN6, MFSD8, CLN8, CTSD.
There is no specific treatment for the condition. However, seizures can be reduced or controlled with anticonvulsant drugs and other proper medications. The physical and occupational therapy can help retain functions as long as possible. The disease slowed down in children who were treated with vitamin C and E along with diets Low in Vitamin A.
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