Batten disease

noun

Bat·​ten disease ˈbat-ᵊn- How to pronounce Batten disease (audio)
variants also Batten's disease
: a fatal genetic neurodegenerative disease that is characterized by seizures, progressive loss of vision, and mental impairment progressing to blindness, paralysis, and dementia and that has an onset during childhood with death typically occurring during the late teens and twenties
also : any of a group of related diseases

Note: Batten disease is a lipofuscinosis marked by the abnormal accumulation of lipofuscin in the brain. The most common juvenile form is inherited as an autosomal recessive trait requiring that both parents pass on a copy of the defective gene on a chromosome other than a sex chromosome.

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Cite this Entry

“Batten disease.” Merriam-Webster.com Medical Dictionary, Merriam-Webster, https://www.merriam-webster.com/medical/Batten%20disease. Accessed 23 Jul. 2024.

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