Definition of Batten disease

Reviewed on 3/29/2021

Batten disease: a rare, fatal genetic condition that typically begins in childhood. It is a form of a group of neurologic disorders called the neuronal ceroid lipofuscinoses, or NCLs. The term Batten disease is sometimes used to refer to all the NCL disorders. Early signs can be vision changes, seizures, clumsiness, or behavior changes. With time, neurologic deficits worsen, and the individual loses sight and motor skills. Cognitive abilities decline and dementia develops with time. The disease is often fatal by the teens or twenties. It is also known as Spielmeyer-Vogt-Sjögren-Batten disease. Batten disease is inherited in an autosomal recessive manner.


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References Batten disease fact sheet.

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