Batten disease (Neuronal Ceroid Lipofuscinoses) is an inherited disorder of the nervous system that usually manifests itself in childhood.
Batten disease is named after the British pediatrician who first described it in 1903. It is one of a group of disorders called neuronal ceroid lipofuscinoses (or NCLs). Although Batten disease is the juvenile form of NCL, most doctors use the same term to describe all forms of NCL.
Early symptoms of Batten disease (or NCL) usually appear in childhood when parents or doctors may notice a child begin to develop vision problems or seizures. In some cases the early signs are subtle, taking the form of personality and behavior changes, difficulties 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 unable to communicate, and presently, it has always been fatal.
Researchers around the world are working hard to find treatments and, ultimately, a cure.