A fatal, inherited disorder of the nervous system that typically begins in childhood. Early symptoms usually appear between the ages of 5 and 10 years when parents or physicians may notice a previously normal child has begun to develop vision problems or seizures. In some cases the early signs are subtle, taking the form of personality and behavior changes, slow learning, clumsiness or stumbling. Over time, affected children suffer mental impairment, worsening seizures and progressive loss of sight and motor skills. Eventually, children with Batten disease become blind, bedridden, and demented; and the disease is often fatal by the late teens or twenties. Although Batten disease originally referred specifically to the juvenile form of NCL (JNCL), the term "Batten disease" is increasingly used by pediatricians to describe all forms of NCL.
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