title: [Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)].
abstract: OBJECTIVE
  The purpose of this paper is to report the case of a patient with Kluver-Bucy syndrome caused by adult-type ceroid lipofuscinosis (Kufs' disease) and to review the literature dealing with the causes of this syndrome.
  
  
  METHODS
  A 38-year-old male examined because of behavioural changes and cognitive impairment. Brain biopsy findings were characteristic of adult-type ceroid lipofuscinosis. This patient fulfilled the criteria of Kufs' disease, since he had mixed clinical features belonging to both type A (neuropsychiatric disorders) and B (aphasia-apraxia-agnosia syndrome) of the disease. The initial symptoms included several clinical features of Klüver-Bucy syndrome (probable visual agnosia, apathy, increased sexual activity, lack of sexual inhibition, hypermetamorphopsia, increased oral behaviour and changes in dietary habits).
  
  
  CONCLUSIONS
  Adult-type ceroid lipofuscinosis is an infrequent clinical entity that is difficult to diagnose owing to the absence of peripheral biological markers and the need to confirm such a diagnosis by means of a histopathological study.
authors.affiliation: Departamento de Medicina-Neurología, Hospital Príncipe de Asturias, Universidad de Alcalá, Madrid, España.
authors.forename: H
authors.initials: H
authors.lastname: Alonso-Navarro
authors.forename: F J
authors.initials: FJ
authors.lastname: Jiménez-Jiménez
authors.forename: I
authors.initials: I
authors.lastname: Puertas-Muñoz
authors.forename: J
authors.initials: J
authors.lastname: Rábano
authors.forename: J G
authors.initials: JG
authors.lastname: de Yébenes
authors.forename: J L
authors.initials: JL
authors.lastname: Sarasa-Corral
mesh_terms: D000328:Adult; D006801:Humans; D020232:Kluver-Bucy Syndrome / Q000175:diagnosis* / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D009472:Neuronal Ceroid-Lipofuscinoses / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology