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5 Rare Neurological Conditions


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Batten Disease

Batten disease is a rare genetic condition that's part of a group of progressive degenerative neurometabolic disorders known as neuronal ceroid lipofuscinoses (NCLS). Currently, it can't be prevented and it is fatal.

The disease causes lipopigments, which are fats and proteins, to abnormally build up in the body's tissues. The disease often begins with vision problems or seizures, followed by significant degeneration of motor coordination and changes in behavior and personality. Seizures and visual impairment worsen as the disease progresses, and sufferers are often blind as well as mentally and physically impaired by the time they lose their battle with the disease.

There are a few types of NCLS disorders -- each type is based on the age symptoms appear rather than the symptoms themselves, which are similar across age groups. Batten disease usually is reserved to describe the condition when it occurs in kids, making itself known by the time the child is between 5 and 10 years old. There is also a form that affects newborns, who die shortly after birth. Other forms may develop during infancy and the toddler years, emerging at about 6 months to 2 years, as well as in early childhood, with symptoms occurring between ages 2 to 4. Adult NCLS appears before age 40, and symptoms are usually less severe than when it develops in children.


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