Batten disease


[Frederick E. Batten, English ophthalmologist, 1865-1918]
The most common of the neuronal ceroid lipofuscinoses (NCLs). Batten disease 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, usually before adolescence. Experimental therapies include treatment with derivatives of hydroxylamine.
SYN: SEE: Spielmeyer-Vogt-Sjogren-Batten disease