The Batten's Disease Syndrome(s) or the Neuronal Ceroid Lipofuscinoses (NCL) are a group of common neurogenetic disorders affecting infants, children, and adults. The incidence of these disorders is estimated to be approximately 1/12,500 births.There are 441,667 carriers of the genetic trait in the U.S. This group of disorders is characterized by intellectual deterioration, visual loss, behavioral changes, the onset of progressively severe seizures, and terminates in death. At the 1987 International Conference on Ceroid Lipofuscinoses several biochemical lesions were examined and discussed critically. Subsequent to the 1987 meeting much new and exciting data has emerged. The 1990 Conference will focus on original and diverse approaches to understanding this disease, namely the localization of the Battens' gene(s) to specific chromosomes, phospholipase A deficiencies in NCL, the characterization and quantitation of NCL specific aldehydes, a serious examination of the role of the accumulation of the DCCD subunits of mitochondrial ATP'ase, the function of protease inhibitors in NCL, correlations of the available NCL animal models with specific types of human NCL, and bone marrow transplantation as an approach to the treatment of NCL patients. Just as the progress of research was advanced by the disclosures and discussions at the 1987 meeting, the 1990 conference should bring together these apparently diverse observations into a cohesive explanation of NCL which will facilitate the development of new approaches to these tragic neurologic disorders. Many NCL families will also attend this conference to hear of the new research findings. The last conference catalyzed the direct involvement of the family support group. The continued interaction of families and scientists will provide a cooperative approach toward the goal of solving the riddle in-NCL. The funds requested are specifically for travel and meeting expenses of the invited speakers.
