Batten disease


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.

REFERENCE: NIH.gov. Batten disease fact sheet.

Read Also:

  • Bathophobia

    Bathophobia: An abnormal and persistent fear of depths. Sufferers from bathophobia experience anxiety even though they realize they are safe from falling into or being consumed by depths. The feared object may be a long, dark hallway, a well or a deep pool or lake. “Bathophobia” is derived from the Greek “bathos” (depth) and “phobos” […]

  • Battered child syndrome

    Battered child syndrome: A disease in which children are physically abused. The battered child syndrome is a form of child abuse. Not until the 19th century were children granted the same legal status as domesticated animals in regard to protection against cruelty and/or neglect. In 1962 the term “battered child syndrome” entered medicine. By 1976 […]

  • Battle fatigue

    The World War II name for what is known today as post-traumatic stress, this is a psychological disorder that develops in some individuals who have had major traumatic experiences (and, for example, have been in a serious accident or through a war). The person is typically numb at first but later has symptoms including depression, […]

  • Bayes theorem

    Bayes theorem: A probability principle set forth by the English mathematician Thomas Bayes (1702-1761). Bayes’ theorem is of value in medical decision-making and some of the biomedical sciences. Bayes’ theorem is employed in clinical epidemiology to determine the probability of a particular disease in a group of people with a specific characteristic on the basis […]

  • Baylisascaris

    Baylisascaris: Infection by the raccoon roundworm. Baylisascaris procyonis is found commonly in raccoons. When infective eggs of this roundworm are ingested by humans, Baylisascaris larvae hatch in the intestine and travel through the organs and muscles. This is the larva migrans syndrome. Infected raccoons shed millions of eggs in their feces. The eggs develop to […]


Disclaimer: Batten disease definition / meaning should not be considered complete, up to date, and is not intended to be used in place of a visit, consultation, or advice of a legal, medical, or any other professional. All content on this website is for informational purposes only.