Batten disease is a fatal, inherited, nervous system disorder that starts in childhood. Another name for this disease is Speilmeyer-Vogt-Sjogren-Batten disease. It is the most common type of neural ceroid lipofuscinoses (NCL) disorder. Symptoms of Batten disease include poor vision and the development of seizures in children between the ages of five and ten years old. Earlier symptoms are not always very noticeable. Some children develop changes in their behavior or personality, become clumsy, and may not learn as fast as other children. As time progresses, the child's mental state deteriorates and seizures become worse. The child's eyesight and motor skills also deteriorate to the point of blindness and they become frenzied and must be confined to a bed. The disease usually results in death by the time the child reaches the latter teen years. However, some people live until their twenties.
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Honor Society of Nursing (STTI) answered
Donna Hill Howes, RN, Administrator, answered
Batten disease is a fatal, inherited disorder of the nervous system that begins in childhood. Early symptoms of this disorder usually appear between the ages of 5 and 10, when parents or physicians may notice that 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 behavioral changes, slow learning, clumsiness, or stumbling. Over time, the 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. Batten disease is often fatal by the late teens or twenties.
Batten disease is named after the British pediatrician who first described it in 1903. Also known as Spielmeyer-Vogt-Sjogren-Batten disease, it is the most common form of a group of disorders called neuronal ceroid lipofuscinoses (or NCL). Although Batten disease is usually regarded as the juvenile form of NCL, some physicians use the term 'Batten disease' to describe all forms of NCL.
This answer is based on source information from the National Institute of Neurological Disorders.