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Batten Disease
A fatal, inherited disease of the nervous system that begins in childhood.
Symptoms include: subtle personality/behavior chagnes slow learning clumsiness stumbling mental impairment seizures that worsen progressive loss of sight and motor skills eventual blindness/deterioration of vision patients become demented completely bedridden
A tragic case of Batten disease inhereted by two boys in the same family
No treatments are known that can stop or reverse the symptoms of this disease. Seaizures can be controlled or reduced by anti- convulsant drugs that prevent or relive the convulsions that are part of seizures.
People who have Batten disease are missing an enzyme that helps break down some fats and proteins. Eventually, the undigested fat and proetin compounds build up in nerve cells, so they stop working.
Recessive disorders like Batten Disease happen when a child recieves to copies of the defective gene, getting one from each parent. If each parent carries one copy of the gene, they are carriers, so their children have a one in four chance of inheriting the disease, a one in four chance of not having the gene at all, and a two in four chance of being a carrier.
Batten disease is rare. It occurs in only 2-4 out of 100,000 live births in the U.S. It is more common in Finland, Sweden, Northern Europe and Newfoundland, Canada.
