Klüver-Bucy syndrome in juvenile neuronal ceroid lipofuscinosis.
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.
Lanska
D J
DJ
Department of Neurology, University of Kentucky Medical Center, Lexington 40536-0084.
Lanska
M J
MJ
D000293:Adolescent; D001321:Autistic Disorder / Q000209:etiology / Q000503:physiopathology; D001921:Brain / Q000503:physiopathology*; D003704:Dementia / Q000209:etiology / Q000503:physiopathology; D007839:Functional Laterality; D006801:Humans; D008297:Male; D009472:Neuronal Ceroid-Lipofuscinoses / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology*; D013064:Speech Disorders / Q000209:etiology / Q000503:physiopathology; D013577:Syndrome