Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)
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. A 38-year-old male examined because of behavioural changes and cognitive impai...
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| Veröffentlicht in: | Revista de neurologiá 2005-01, Vol.40 (2), p.93-98 |
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| container_title | Revista de neurologiá |
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| creator | Alonso-Navarro, H Jiménez-Jiménez, F J Puertas-Muñoz, I Rábano, J de Yébenes, J G Sarasa-Corral, J L |
| description | 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.
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).
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. |
| doi_str_mv | 10.33588/rn.4002.2004538 |
| format | Article |
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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).
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.</description><identifier>ISSN: 0210-0010</identifier><identifier>DOI: 10.33588/rn.4002.2004538</identifier><identifier>PMID: 15712163</identifier><language>spa</language><publisher>Spain</publisher><subject>Adult ; Humans ; Kluver-Bucy Syndrome - diagnosis ; Kluver-Bucy Syndrome - etiology ; Kluver-Bucy Syndrome - pathology ; Kluver-Bucy Syndrome - physiopathology ; Male ; Neuronal Ceroid-Lipofuscinoses - complications ; Neuronal Ceroid-Lipofuscinoses - diagnosis ; Neuronal Ceroid-Lipofuscinoses - pathology ; Neuronal Ceroid-Lipofuscinoses - physiopathology</subject><ispartof>Revista de neurologiá, 2005-01, Vol.40 (2), p.93-98</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/15712163$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Alonso-Navarro, H</creatorcontrib><creatorcontrib>Jiménez-Jiménez, F J</creatorcontrib><creatorcontrib>Puertas-Muñoz, I</creatorcontrib><creatorcontrib>Rábano, J</creatorcontrib><creatorcontrib>de Yébenes, J G</creatorcontrib><creatorcontrib>Sarasa-Corral, J L</creatorcontrib><title>Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)</title><title>Revista de neurologiá</title><addtitle>Rev Neurol</addtitle><description>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.
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).
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.</description><subject>Adult</subject><subject>Humans</subject><subject>Kluver-Bucy Syndrome - diagnosis</subject><subject>Kluver-Bucy Syndrome - etiology</subject><subject>Kluver-Bucy Syndrome - pathology</subject><subject>Kluver-Bucy Syndrome - physiopathology</subject><subject>Male</subject><subject>Neuronal Ceroid-Lipofuscinoses - complications</subject><subject>Neuronal Ceroid-Lipofuscinoses - diagnosis</subject><subject>Neuronal Ceroid-Lipofuscinoses - pathology</subject><subject>Neuronal Ceroid-Lipofuscinoses - physiopathology</subject><issn>0210-0010</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2005</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo1kLtOwzAYRj2AaCnsTMgTlyHlt53YyUgrbmolFpiDr8LIiUOcIOXd2HgxKlGmTzo6OsOH0BmBJWNFWd707TIHoEsKkBesPEBzoAQyAAIzdJzSx46zvIIjNCOFIJRwNkdvm_Dz_WX7bDXqCaepNX1sLJYJD-8W-9YPXoYdb7ohNjg6LM0YhmyYOou17aM3OPguujFp38bkE77ajC5dYuOTlclen6BDJ0Oyp_tdoNf7u5f1Y7Z9fnha326zjrBqyAQDqyugyoDgVApe2bzUQinGNSW6LIzmJROGS6NYVTgqHBDBlVDaEe0KtkAXf92uj5-jTUPd-KRtCLK1cUw1FzmrBOU78Xwvjqqxpu5638h-qv8_Yb_G_WNo</recordid><startdate>20050116</startdate><enddate>20050116</enddate><creator>Alonso-Navarro, H</creator><creator>Jiménez-Jiménez, F J</creator><creator>Puertas-Muñoz, I</creator><creator>Rábano, J</creator><creator>de Yébenes, J G</creator><creator>Sarasa-Corral, J L</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>20050116</creationdate><title>Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)</title><author>Alonso-Navarro, H ; Jiménez-Jiménez, F J ; Puertas-Muñoz, I ; Rábano, J ; de Yébenes, J G ; Sarasa-Corral, J L</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p139t-730ec902bd0762a769e48c7bb36c21c85dc6837d6adb395f27f0176b7bcf1cf53</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>spa</language><creationdate>2005</creationdate><topic>Adult</topic><topic>Humans</topic><topic>Kluver-Bucy Syndrome - diagnosis</topic><topic>Kluver-Bucy Syndrome - etiology</topic><topic>Kluver-Bucy Syndrome - pathology</topic><topic>Kluver-Bucy Syndrome - physiopathology</topic><topic>Male</topic><topic>Neuronal Ceroid-Lipofuscinoses - complications</topic><topic>Neuronal Ceroid-Lipofuscinoses - diagnosis</topic><topic>Neuronal Ceroid-Lipofuscinoses - pathology</topic><topic>Neuronal Ceroid-Lipofuscinoses - physiopathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Alonso-Navarro, H</creatorcontrib><creatorcontrib>Jiménez-Jiménez, F J</creatorcontrib><creatorcontrib>Puertas-Muñoz, I</creatorcontrib><creatorcontrib>Rábano, J</creatorcontrib><creatorcontrib>de Yébenes, J G</creatorcontrib><creatorcontrib>Sarasa-Corral, J L</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><jtitle>Revista de neurologiá</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Alonso-Navarro, H</au><au>Jiménez-Jiménez, F J</au><au>Puertas-Muñoz, I</au><au>Rábano, J</au><au>de Yébenes, J G</au><au>Sarasa-Corral, J L</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)</atitle><jtitle>Revista de neurologiá</jtitle><addtitle>Rev Neurol</addtitle><date>2005-01-16</date><risdate>2005</risdate><volume>40</volume><issue>2</issue><spage>93</spage><epage>98</epage><pages>93-98</pages><issn>0210-0010</issn><abstract>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.
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).
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.</abstract><cop>Spain</cop><pmid>15712163</pmid><doi>10.33588/rn.4002.2004538</doi><tpages>6</tpages></addata></record> |
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| source | MEDLINE; EZB-FREE-00999 freely available EZB journals |
| subjects | Adult Humans Kluver-Bucy Syndrome - diagnosis Kluver-Bucy Syndrome - etiology Kluver-Bucy Syndrome - pathology Kluver-Bucy Syndrome - physiopathology Male Neuronal Ceroid-Lipofuscinoses - complications Neuronal Ceroid-Lipofuscinoses - diagnosis Neuronal Ceroid-Lipofuscinoses - pathology Neuronal Ceroid-Lipofuscinoses - physiopathology |
| title | Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease) |
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