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[Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)].

AbstractAIMS:
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.
AuthorsH Alonso-Navarro, F J Jiménez-Jiménez, I Puertas-Muñoz, J Rábano, J G de Yébenes, J L Sarasa-Corral
JournalRevista de neurologia (Rev Neurol) 2005 Jan 16-31 Vol. 40 Issue 2 Pg. 93-8 ISSN: 0210-0010 [Print] Spain
Vernacular TitleSíndrome de Klüver-Bucy como manifestación inicial de ceroidolipofuscinosis del adulto (enfermedad de Kufs).
PMID15712163 (Publication Type: Case Reports, English Abstract, Journal Article)
Topics
  • Adult
  • Humans
  • Kluver-Bucy Syndrome (diagnosis, etiology, pathology, physiopathology)
  • Male
  • Neuronal Ceroid-Lipofuscinoses (complications, diagnosis, pathology, physiopathology)

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