Batten disease is a neurological disorder that affects individuals with genetic issues. This medical condition shows its early signs during childhood, and it slowly destroys the nervous system of the carrier. Unfortunately, the illness is fatal and modern medicine has not developed a reliable treatment yet.
The first signs of Batten disease appear within the first 10 years of life. During this period the child may develop various medical conditions that affect his vision, his natural movement or his speech. The main obstacle that many doctors encounter is that the symptoms are difficult to identify right away. More than often, the patient gets a definite diagnose once the illness has already settled in.
Other symptoms of Batten disease include mood changes, personality issues and learning deficiencies. Teeth grinding, clumsiness and decreased body fat are also common for children who suffer from this illness. These subtle signs show just how easy it is for parents and physicians to determine whether a kid is developing a fatal neurological disorder.
Batten disease is cruel for its victims. Children that develop this condition between 6 months and 2 years old are likely to die before their sixth birthday. Kids between 2 and 4 years old do not survive past the age of 10. These cases are the hardest to identify and treat for doctors because the early signs do not point to neurological issues specifically.
Children that develop Batten disease between 4 and 10 years old may live until they reach their mid 20s. Nevertheless, their lifestyle is strongly affected by the continuous deterioration of their neurons. Rare cases of Batten disease have seen adults between 25 and 45 years old developing this illness as well. In these unusual situations, the patients may live a normal lifespan, but with serious and constant damaging of their neurological system.
Causes and origins
Batten disease is a neurodegenerative disorder that is inherited through an autosomal recessive process. This means that even if the parents are not carriers of the destructive gene, they can still transfer it to one or more of their children. Usually, unaffected carrier parents have a 25% chance of transmitting the gene in its active form to each of their first three children. Their fourth born has a 100% chance of developing Batten disease anytime during his childhood.
This neurological disorder was first identified by the British pediatrician Frederick Batten in 1903, but it has not been recorded as an official disease until the end of the 20th century. Even today, there are medical schools that group it together with other conditions that affect children in their early infancy.
Research and treatment
The medical field puts a lot of effort in developing a permanent cure for Batten disease. Unfortunately, the scientific research has not produced any suitable treatments so far. The FDA has approved only a small number of remedies that patients can use to alleviate some of the issues that appear with the illness, and which make the neurological breakdown more bearable.