Behaviour problems in Angelman syndrome
Angelman syndrome (AS) is a genetic disorder that is associated with a deletion on chromosome 15, and is characterized by abnormalities or impairments in neurological, motor and intellectual functioning. While behaviour problems have been reported iri clients with AS, relatively little is known abou...
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| Veröffentlicht in: | Journal of intellectual disability research 1995-04, Vol.39 (2), p.97-106 |
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| container_title | Journal of intellectual disability research |
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| creator | Summers, J. A. Allison, D. B. Lynch, P. S. Sandier, L. |
| description | Angelman syndrome (AS) is a genetic disorder that is associated with a deletion on chromosome 15, and is characterized by abnormalities or impairments in neurological, motor and intellectual functioning. While behaviour problems have been reported iri clients with AS, relatively little is known about their developmental course and outcome. In this study, data on the nature and prevalence of behaviour problems among clients with AS were gathered from two sources: (i) a review of published case reports; and (2) parent responses to a survey of behaviour problems in a small (n= 11) sample of children with AS. Data from both sources showed that behaviour problems were present in males and females of all ages, and included language deficits, excessive laughter, hyperactivity, short attention span, problems with eating and sleeping, aggression, noncompliance, mouthing of objects, tantrums, and repetitive and stereotyped behaviour. Identification and treatment of severe behaviour problems in clients with AS may improve their adaptive functioning. |
| doi_str_mv | 10.1111/j.1365-2788.1995.tb00477.x |
| format | Article |
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A. ; Allison, D. B. ; Lynch, P. S. ; Sandier, L.</creator><creatorcontrib>Summers, J. A. ; Allison, D. B. ; Lynch, P. S. ; Sandier, L.</creatorcontrib><description>Angelman syndrome (AS) is a genetic disorder that is associated with a deletion on chromosome 15, and is characterized by abnormalities or impairments in neurological, motor and intellectual functioning. While behaviour problems have been reported iri clients with AS, relatively little is known about their developmental course and outcome. In this study, data on the nature and prevalence of behaviour problems among clients with AS were gathered from two sources: (i) a review of published case reports; and (2) parent responses to a survey of behaviour problems in a small (n= 11) sample of children with AS. Data from both sources showed that behaviour problems were present in males and females of all ages, and included language deficits, excessive laughter, hyperactivity, short attention span, problems with eating and sleeping, aggression, noncompliance, mouthing of objects, tantrums, and repetitive and stereotyped behaviour. Identification and treatment of severe behaviour problems in clients with AS may improve their adaptive functioning.</description><identifier>ISSN: 0964-2633</identifier><identifier>EISSN: 1365-2788</identifier><identifier>DOI: 10.1111/j.1365-2788.1995.tb00477.x</identifier><identifier>PMID: 7787388</identifier><identifier>CODEN: JIDREN</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Adolescent ; Adult ; Aggression ; Angelman syndrome ; Angelman Syndrome - complications ; Angelman Syndrome - genetics ; Angelman Syndrome - psychology ; Behavioural problems ; Biological and medical sciences ; Child ; Child, Preschool ; Feeding Behavior ; Female ; Humans ; Infant ; Language Disorders - etiology ; Laughter ; Male ; Malformations of the nervous system ; Medical sciences ; Neurology ; Patients ; Seizures - etiology ; Sleep Wake Disorders - etiology ; Stereotyped Behavior</subject><ispartof>Journal of intellectual disability research, 1995-04, Vol.39 (2), p.97-106</ispartof><rights>1995 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4667-ec016a709f2ff54d4d69b2c9e38f7154e89eb74de57cac73f48b9727726dbd783</citedby><cites>FETCH-LOGICAL-c4667-ec016a709f2ff54d4d69b2c9e38f7154e89eb74de57cac73f48b9727726dbd783</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27915,27916,30991</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=3470936$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/7787388$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Summers, J. A.</creatorcontrib><creatorcontrib>Allison, D. B.</creatorcontrib><creatorcontrib>Lynch, P. S.</creatorcontrib><creatorcontrib>Sandier, L.</creatorcontrib><title>Behaviour problems in Angelman syndrome</title><title>Journal of intellectual disability research</title><addtitle>J Intellect Disabil Res</addtitle><description>Angelman syndrome (AS) is a genetic disorder that is associated with a deletion on chromosome 15, and is characterized by abnormalities or impairments in neurological, motor and intellectual functioning. While behaviour problems have been reported iri clients with AS, relatively little is known about their developmental course and outcome. In this study, data on the nature and prevalence of behaviour problems among clients with AS were gathered from two sources: (i) a review of published case reports; and (2) parent responses to a survey of behaviour problems in a small (n= 11) sample of children with AS. Data from both sources showed that behaviour problems were present in males and females of all ages, and included language deficits, excessive laughter, hyperactivity, short attention span, problems with eating and sleeping, aggression, noncompliance, mouthing of objects, tantrums, and repetitive and stereotyped behaviour. Identification and treatment of severe behaviour problems in clients with AS may improve their adaptive functioning.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aggression</subject><subject>Angelman syndrome</subject><subject>Angelman Syndrome - complications</subject><subject>Angelman Syndrome - genetics</subject><subject>Angelman Syndrome - psychology</subject><subject>Behavioural problems</subject><subject>Biological and medical sciences</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Feeding Behavior</subject><subject>Female</subject><subject>Humans</subject><subject>Infant</subject><subject>Language Disorders - etiology</subject><subject>Laughter</subject><subject>Male</subject><subject>Malformations of the nervous system</subject><subject>Medical sciences</subject><subject>Neurology</subject><subject>Patients</subject><subject>Seizures - etiology</subject><subject>Sleep Wake Disorders - etiology</subject><subject>Stereotyped Behavior</subject><issn>0964-2633</issn><issn>1365-2788</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1995</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>7QJ</sourceid><recordid>eNqVkEtr3DAUhUVoSSaPnxAYQmlWdiXrcaVCF3k1TckDkpRCNkKWr1tP_UilmWTm39dmzGxLtLmLc87VuR8hR4ymrH-fZinjSiYZaJ0yY2Q6zykVAOlyi0w20jsyoUaJJFOc75DdGGeUUsWE2ibbABq41hNyfIq_3UvVLcL0OXR5jU2cVu30pP2FdePaaVy1Rega3CfvS1dHPBjnHvnx9eLx7FtyfXd5dXZynXihFCToKVMOqCmzspSiEIUyeeYNcl0CkwK1wRxEgRK888BLoXMDGUCmirwAzffIx_Xevs3fBca5barosa5di90iWgAumKTwX6MEMFJT1Rs_r40-dDEGLO1zqBoXVpZRO-C0MzswswMzO-C0I0677MOH4y-LvMFiEx359fqHUXfRu7oMrvVV3Ni46FHwocOXte21qnH1hgL2-9W9GY5N1vkqznG5ybvwxyrgIO3P20t78yCfzk8fbq3i_wCcVJ-4</recordid><startdate>199504</startdate><enddate>199504</enddate><creator>Summers, J. A.</creator><creator>Allison, D. B.</creator><creator>Lynch, P. S.</creator><creator>Sandier, L.</creator><general>Blackwell Publishing Ltd</general><general>Blackwell</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7QJ</scope><scope>7X8</scope></search><sort><creationdate>199504</creationdate><title>Behaviour problems in Angelman syndrome</title><author>Summers, J. A. ; Allison, D. B. ; Lynch, P. S. ; Sandier, L.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4667-ec016a709f2ff54d4d69b2c9e38f7154e89eb74de57cac73f48b9727726dbd783</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1995</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aggression</topic><topic>Angelman syndrome</topic><topic>Angelman Syndrome - complications</topic><topic>Angelman Syndrome - genetics</topic><topic>Angelman Syndrome - psychology</topic><topic>Behavioural problems</topic><topic>Biological and medical sciences</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Feeding Behavior</topic><topic>Female</topic><topic>Humans</topic><topic>Infant</topic><topic>Language Disorders - etiology</topic><topic>Laughter</topic><topic>Male</topic><topic>Malformations of the nervous system</topic><topic>Medical sciences</topic><topic>Neurology</topic><topic>Patients</topic><topic>Seizures - etiology</topic><topic>Sleep Wake Disorders - etiology</topic><topic>Stereotyped Behavior</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Summers, J. A.</creatorcontrib><creatorcontrib>Allison, D. B.</creatorcontrib><creatorcontrib>Lynch, P. S.</creatorcontrib><creatorcontrib>Sandier, L.</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Applied Social Sciences Index & Abstracts (ASSIA)</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of intellectual disability research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Summers, J. A.</au><au>Allison, D. B.</au><au>Lynch, P. S.</au><au>Sandier, L.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Behaviour problems in Angelman syndrome</atitle><jtitle>Journal of intellectual disability research</jtitle><addtitle>J Intellect Disabil Res</addtitle><date>1995-04</date><risdate>1995</risdate><volume>39</volume><issue>2</issue><spage>97</spage><epage>106</epage><pages>97-106</pages><issn>0964-2633</issn><eissn>1365-2788</eissn><coden>JIDREN</coden><abstract>Angelman syndrome (AS) is a genetic disorder that is associated with a deletion on chromosome 15, and is characterized by abnormalities or impairments in neurological, motor and intellectual functioning. While behaviour problems have been reported iri clients with AS, relatively little is known about their developmental course and outcome. In this study, data on the nature and prevalence of behaviour problems among clients with AS were gathered from two sources: (i) a review of published case reports; and (2) parent responses to a survey of behaviour problems in a small (n= 11) sample of children with AS. Data from both sources showed that behaviour problems were present in males and females of all ages, and included language deficits, excessive laughter, hyperactivity, short attention span, problems with eating and sleeping, aggression, noncompliance, mouthing of objects, tantrums, and repetitive and stereotyped behaviour. Identification and treatment of severe behaviour problems in clients with AS may improve their adaptive functioning.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>7787388</pmid><doi>10.1111/j.1365-2788.1995.tb00477.x</doi><tpages>10</tpages></addata></record> |
| fulltext | fulltext |
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| ispartof | Journal of intellectual disability research, 1995-04, Vol.39 (2), p.97-106 |
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| language | eng |
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| source | MEDLINE; Applied Social Sciences Index & Abstracts (ASSIA); Education Source |
| subjects | Adolescent Adult Aggression Angelman syndrome Angelman Syndrome - complications Angelman Syndrome - genetics Angelman Syndrome - psychology Behavioural problems Biological and medical sciences Child Child, Preschool Feeding Behavior Female Humans Infant Language Disorders - etiology Laughter Male Malformations of the nervous system Medical sciences Neurology Patients Seizures - etiology Sleep Wake Disorders - etiology Stereotyped Behavior |
| title | Behaviour problems in Angelman syndrome |
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