Promoting support to all those affected on this Island of ours and beyond. Registered In Ireland CHY 19226
Batten disease is an inherited disorder of the nervous system that usually manifests itself in childhood. Batten disease is named after the British paediatrician 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 behaviour changes, delayed speech, slow learning, clumsiness or stumbling. Over time, affected children suffer mental impairment, worsening seizures, and progressive loss of sight and motor skills. Children become totally disabled and eventually die. Batten disease is not contagious nor, at this time, preventable. To date it has always been fatal. There are four main types of NCL; Infantile, Late infantile, Juvenile and a very rare form, Adult NCL. Symptoms vary with each child. In some, the disease progresses more quickly and in some slower. Early symptoms of Batten disease are confusing and not easily recognised even by medical personnel. Batten disease is rarely diagnosed immediately because of the variability in symptoms and age of onset. Children are often mistakenly thought to have epilepsy or a form of mental retardation. Adults are sometimes labelled schizophrenics. This can be a difficult and frustrating time for all concerned.
