THE NEUROPSYCHOLOGY OF KUFS-DISEASE - A CASE OF ATYPICAL EARLY-ONSET DEMENTIA

Adult Neuronal Ceroid Lipofuscinosis (Adult NCL), also known as Kuf's Disease, is a rare progressive encephalopathic disease that results in dementia. This report describes the presentation, diagnosis, neuroana tomy, and management of Adult NCL. A neuropsychological case study of Adult NCL is presented, emphasizing early onset of psychiatric symptom s, motor involvement, and severe diffuse cognitive impairment. The pat ient's neuropsychological course is documented over a 2-year period, w ith findings interpreted within the context of current models of corti cal and subcortical dementia. The biopsychosocial impact of misdiagnos is and pharmaceutical management are discussed. (C) 1997 National Acad emy of Neuropsychology.