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Klüver-Bucy Syndrome in Juvenile Neuronal Ceroid Lipofuscinosis

Department of Neurology, University of Kentucky Medical Center, Preventive Medicine and Environmental Health, University of Kentucky Medical Center, Sanders-Brown Center on Aging, University of Kentucky Mecical Center, Alzheimer's Disease Research Center University of Kentucky Medical Center, Neurology Service Department of Veterans Affairs Medical Center, Lexington, KY

Mary Jo Lanska, MD, MS

Department of Neurology, University of Kentucky Medical Center, Department of Pediatrics, University of Kentucky Medical Center

Although Klüver-Bucy syndrome in adults is commonly associated with neurodegenerative conditions, Klüver-Bucy syndrome in children has been recognized almost exclusively in association with acute bitemporal injury or dysfunction. We report a child with juvenile neuronal ceroid lipofuscinosis, who developed dementia, childhood-onset autistic disorder, and Klüver-Bucy syndrome. The behavioral features of this case are compared with those of previously reported cases of juvenile neuronal ceroid lipofuscinosis and with previous reports of Klüver-Bucy syndrome in children and adults. (J Child Neurol 1994;9;67-69).

Journal of Child Neurology, Vol. 9, No. 1, 67-69 (1994)
DOI: 10.1177/088307389400900117


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