The neuropsychology of Kuf's 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, neuroanatomy, and management of Adult NCL. A neuropsychological case study of Adult NCL is presented, emphasizing early onset of psychiatric symptoms, motor involvement, and severe diffuse cognitive impairment. The patient's neuropsychological course is documented over a 2-year period, with findings interpreted within the context of current models of cortical and subcortical dementia. The biopsychosocial impact of misdiagnosis and pharmaceutical management are discussed.