Special Issue Article Neurobehavioral Features and Natural History of Juvenile Neuronal Ceroid

Juvenile neuronal ceroid lipofuscinosis is a childhood-onset neurodegenerative disease with prominent symptoms comprising a pediatric dementia syndrome: intellectual decline,mood and behavioral impairments, and loss of adaptive skills.We review the history of neurobehavioral features in juvenile neuronal ceroid lipofuscinosis and the work of the University of Rochester Batten Center to characterize the extent and progression of neurobehavioral symptoms over the disease course, and discuss the relevance of neuro-behavioral studies as an aid to understanding the clinical phenotype of juvenile Batten disease and potential targets for intervention