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.
Department of Psychology, Memorial Medical Center, Springfield, IL 62781, USA.
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JH
Hinkebein
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Callahan