Abstract | AIMS: 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. CASE REPORT: 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.
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Authors | H Alonso-Navarro, F J Jiménez-Jiménez, I Puertas-Muñoz, J Rábano, J G de Yébenes, J L Sarasa-Corral |
Journal | Revista de neurologia
(Rev Neurol)
2005 Jan 16-31
Vol. 40
Issue 2
Pg. 93-8
ISSN: 0210-0010 [Print] Spain |
Vernacular Title | Síndrome de Klüver-Bucy como manifestación inicial de ceroidolipofuscinosis del adulto (enfermedad de Kufs). |
PMID | 15712163
(Publication Type: Case Reports, English Abstract, Journal Article)
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Topics |
- Adult
- Humans
- Kluver-Bucy Syndrome
(diagnosis, etiology, pathology, physiopathology)
- Male
- Neuronal Ceroid-Lipofuscinoses
(complications, diagnosis, pathology, physiopathology)
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