[Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)].
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.
Alonso-Navarro
H
H
Departamento de Medicina-Neurología, Hospital Príncipe de Asturias, Universidad de Alcalá, Madrid, España.
Jiménez-Jiménez
F J
FJ
Puertas-Muñoz
I
I
Rábano
J
J
de Yébenes
J G
JG
Sarasa-Corral
J L
JL
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