Neurobehavioral Features and Natural History of Juvenile Neuronal Ceroid Lipofuscinosis (Batten Disease). (September 2013)

• Record Type: Journal Article
• Authors: Adams, Heather R.; Mink, Jonathan W.
• Is Part Of: Journal of child neurology. Volume 28:Number 9(2013)
• Page Start: 1128
• Page End: 1136
• Publication Date: 2013-09
• Subjects: behavior -- cognition -- neuropsychology -- quality of life -- Unified Batten Disease Rating Scale; Nervous system -- Diseases -- Periodicals; 618.928
• DOI: 10.1177/0883073813494813
• Languages: English
• ISSNs: 0883-0738

Abstract

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 neurobehavioral studies as an aid to understanding the clinical phenotype of juvenile Batten disease and potential targets for intervention.