Nota Clínica

Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs’ disease)

H. Alonso-Navarro, F. J. Jiménez-Jiménez, I. Puertas-Muñoz, J. Rábano, J.G. de Yébenes, J.L. Sarasa-Corral [REV NEUROL 2005;40:93-98] PMID: 15712163 DOI: https://doi.org/10.33588/rn.4002.2004538

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Volumen 40 | Number 02 | Nº of views of the article 22.945 | Nº of PDF downloads 923 | Article publication date 16/01/2005

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ABSTRACT

AIMS. The purpose of this paper is to report the case of a patient with Klüver-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. KeywordsCeroid lipofuscinosis Etiology Klüver-Bucy syndrome Kufs’ disease

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Nota Clínica

Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs’ disease)