(Băt′ĕn)
[Pronunciation]
[Frederick E. Batten, English ophthalmologist, 1865–1918]
The most common of the neuronal ceroid lipofuscinoses (NCLs).
This eponym is sometimes used as a synonym for the entire class of NCLs. The disease is an autosomal recessive, neurodegenerative disorder that results from the excessive accumulation of lipid-protein complexes in the brain and eye. Early symptoms include the sudden onset of visual impairment and seizures in childhood. Regression of developmental milestones and dementia precede premature death.