Neuronal ceroid lipofuscinosis also known as the Batten disease results from excessive accumulation of lipopygments in body’s tissue.

This caused by lack one of several enzymes necessary for the lipopygments normal breakdown.

The lipopygments are called lipofuscin, also known as autofluorescent lysosomal storage bodies.

  The lipopygment lipofuscin in case of Neuronal ceroid lipofuscinosis builds up in the neural cells and some organs, such as liver, spleen, kidneys etc.

This storage causes neuronal loss, cortical atrophy, and cerebellar and retinal degeneration resulting in seizures, progressive deterioration of cognition (dementia), motor function impairment (involuntary movements, myoclonus, ataxia, spasticity) and blindness.