Medical Definition of Batten disease

  • Medical Author:
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

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.

Reviewed on 12/21/2018

REFERENCE: Batten disease fact sheet.

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