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Williams Syndrome (updated March 2003)

What information do you have on Williams syndrome ?

The following information is from the Williams Syndrome Association.

Williams syndrome is a rare genetic condition (estimated to occur in 1/20,000 births) that causes medical and developmental problems. It was first recognized as a distinct entity in 1961. It is present at birth, and affects males and females equally. It can occur in all ethnic groups and has been identified in countries throughout the world.

Williams syndrome is not caused by anything the parents did or did not do either before or during pregnancy. We know that most individuals with Williams syndrome are missing genetic material on chromosome #7 including the gene that makes the protein elastin (a protein which provides strength and elasticity to vessel walls.) It is likely that the elastin gene deletion accounts for many of the physical features of Williams syndrome. Some medical and developmental problems are probably caused by deletions of additional genetic material near the elastin gene on chromosome #7. The extent of these deletions may vary among individuals. In most families the child with Williams syndrome is the only one to have the condition in his or her entire extended family. However, the individual with Williams syndrome has a 50% chance of passing the disorder on to each of his or her children.

Individuals with Williams syndrome have a very endearing personality. They have a unique strength in their expressive language skills, and are extremely polite. They are typically unafraid of strangers and show a greater interest in contact with adults than with their peers.

Most people with Williams syndrome have some degree of intellectual disability. Young children with Williams syndrome often experience developmental delays; milestones such as walking, talking and toilet training are often achieved somewhat later than is considered normal. Distractibility is a common problem in mid-childhood, which appears to get better as the children get older.

Older children and adults with Williams syndrome often demonstrate intellectual "strengths and weaknesses." There are some intellectual areas (such as speech, long term memory, and social skills) in which performance is quite strong, while other intellectual areas (such as fine motor and spatial relations) are significantly deficient.

The vast majority of adults with Williams syndrome master self-help skills and complete academic and/or vocational school. They are employed in a variety of settings (ranging from supervised to independent jobs). Many adults with Williams syndrome live with their parents; others live in supervised apartments and some are able to live on their own.

Following are links to related Internet resources and Internet discussion groups, as well as selected citations from the ERIC database and the search terms we used to find the citations.

You can search the ERIC database yourself on the Internet through either of the following web sites:

NOTE: The remainder of this file includes citations on Williams syndrome from the ERIC database, whose primary focus is education. While the ERIC database contains information on some medical disorders, you may want to search a medical database such as MEDLINE/MEDLARS at the National Library of Medicine, http://www.nlm.nih.gov, or PubMed http://www.ncbi.nlm.nih.gov/PubMed/ or MedWeb at Emory University, http://WWW.MedWeb.Emory.Edu/MedWeb/.

ERIC Citations

The full text of citations beginning with an ED number (for example, EDxxxxxx) is available:

  • In microfiche collections worldwide; to find your nearest ERIC Resource Collection, point your web browser to: http://ericae.net/derc.htm.
  • For a fee through the ERIC Document Reproduction Service (EDRS): http://edrs.com, service@edrs.com, or 1.800.443.ERIC. (no longer available)

The full text of citations beginning with an EJ number (for example, EJxxxxxx) is available for a fee from:

ERIC Search Terms Used

Williams syndrome

EJ576109 PS528618
Syntax and Morphology in Williams Syndrome.
Clahsen, Harald; Almazan, Mayella
Cognition; v68 n3 p167-98 Sep 1998
ISSN: ISSN-0010-0277
Language: English
Document Type: JOURNAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJJUL1999
Investigated four cases of English-speaking children with Williams Syndrome (WS), a neuro-developmental disorder characterized by an unusual fractionation of language abilities. Found that, despite low IQ, subjects performance on syntactic tasks and on regular inflection is not impaired, suggesting a distinction between a computation system and an associative memory system for language in children with WS.
Descriptors: *Children; Language Impairments; *Language Processing; *Morphology (Languages); *Syntax;
Identifiers: *Williams Syndrome

EJ627909 EC627467
Adaptive Behavior of 4- through 8-Year-Old Children with Williams Syndrome.
Mervis, Carolyn B.; Klein-Tasman, Bonita P.; Mastin, Michelle E.
American Journal on Mental Retardation; v106 n1 p82-93 Jan 2001
ISSN: ISSN-0895-8017
Language: English
Document Type: JOURNAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJDEC2001
This study assessed the behavior of 41 4-through 8-year-olds with Williams syndrome. As expected, socialization and communication were relative strengths, whereas daily living skills and motor skills were relative weaknesses. Within socialization, interpersonal skills were stronger than play/leisure or coping skills. Adaptive behavior was not related to chronological age.
Descriptors: *Adjustment (to Environment); *Chronological Age; Communication Skills; Daily Living Skills; Interpersonal Competence; *Mental Retardation; Perceptual Motor Coordination; *Psychological Characteristics; Socialization; Young Children;
Identifiers: *Williams Syndrome

EJ627908 EC627466
Longitudinal Course of Behavioral and Emotional Problems in Williams Syndrome.
Einfeld, Stewart L.; Tonge, Bruce J.; Rees, Vaughan W.
American Journal on Mental Retardation; v106 n1 p73-81 Jan 2001
ISSN: ISSN-0895-8017
Language: English
Document Type: JOURNAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJDEC2001
A follow-up study of behavior and emotional problems in 53 young people with Williams syndrome 5 years after first assessment found substantial persistence in the overall level of behavior and emotional problems. Comparison with young people with mental retardation due to other causes found Williams subjects had significantly higher overall behavioral and emotional problems, communication disturbance, and anxiety.
Descriptors: Adolescents; Anxiety; *Behavior Problems; Children; Communication Disorders; *Emotional Problems; Follow-up Studies; *Incidence; *Mental Retardation;
Identifiers: *Williams Syndrome

EJ623095 EC626818
Assessment of the Influence of Background Noise on Escape-Maintained Problem Behavior and Pain Behavior in a Child with Williams Syndrome.
O'Reilly, Mark F.; Lacey, Claire; Lancioni, Giulio E.
Journal of Applied Behavior Analysis; v33 n4 p511-14 Win 2000
ISSN: ISSN-0021-8855
Language: English
Document Type: JOURNAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJSEP2001
A study examined the influence of background noise on levels of problem behavior and pain behavior under functional analysis conditions for a 5-year-old with Williams syndrome and hyperacusis. When the child was fitted with earplugs, there were substantial decreases in both problem and pain behavior under the background noise condition.
Descriptors: Auditory Stimuli; *Behavior Problems; *Environmental Influences; *Mental Retardation; *Noise (Sound); Outcomes of Treatment; *Pain; Young Children;
Identifiers: *Functional Behavioral Assessment; Williams Syndrome

EJ653469 CS763139
Investigating Reading Development in Atypical Populations: The Case of Williams Syndrome.
Laing, Emma
Reading and Writing: An Interdisciplinary Journal; v15 n5-6 p575-87 Sep 2002
ISSN: ISSN-0922-4777
Language: English
Journal Announcement: CIJFEB2003
Highlights the methodological issues surrounding research on reading in atypical populations and discusses the issue of comparison groups. Reviews evidence from studies that have described the reading profile observed in Williams syndrome. Advocates a more dynamic developmental approach to the study of reading in atypical populations.
Descriptors: Elementary Secondary Education; Learning Disabilities; Literature Reviews; *Reading Ability; Reading Achievement; *Reading Diagnosis; *Reading Instruction; *Reading Research;
Identifiers: Phonological Awareness; *Williams Syndrome

EJ626507 FL531473
Past Tense Formation in Williams Syndrome.
Thomas, Michael S. C.; Grant, Julia; Barham, Zita; Gsodl, Marisa; Laing, Emma; Lakusta, Laura; Tyler, Lorraine K.; Grice, Sarah; Paterson, Sarah; Karmiloff-Smith, Annette
Language and Cognitive Processes; v16 n2-3 p143-76 Apr-Jun 2001
ISSN: ISSN-0169-0965
Language: English
Document Type: JOURNAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJNOV2001
Compared the performance of participants with Williams Syndrome on two past tense elicitation tasks with that of four typically-developing control groups. Results were consistent with the hypothesis that the Williams Syndrome language system is delayed, because it developed under different constraints.
Descriptors: *Cognitive Processes; Comparative Analysis; *Language Impairments; Phonology; Syntax; Task Analysis; *Tenses (Grammar);
Identifiers: *Williams Syndrome
EJ650463 EC630394
Teaching Creative Dramatics to Young Adults with Williams Syndrome.
Tieso, Carol L.
TEACHING Exceptional Children; v34 n6 p32-38 Jul-Aug 2002
ISSN: ISSN-0040-0599
Language: English
Document Type: JOURNAL ARTICLES (080); REPORTS (141)
Journal Announcement: CIJDEC2002
This article describes a university affiliated summer program which provided 16 young adults with Williams Syndrome with a creative dramatics program highlighting their language and musical talents. The article discusses the characteristic strengths and weaknesses of people with Williams syndrome, meeting students' interests and learning styles, and scaffolding for optimal learning.
Descriptors: Congenital Impairments; Dramatics; *Creative Development; *Mental Retardation; Postsecondary Education; Scaffolding (Teaching Technique); Secondary Education; Summer Programs; Symptoms (Individual Disorders); *Talent Development; Young Adults;
Identifiers: *Williams Syndrome

EJ608633 PS530719
A Componential View of Theory of Mind: Evidence from Williams Syndrome.
Tager-Flusberg, Helen; Sullivan, Kate
Cognition; v76 n1 p59-89 Jul 14 2000
ISSN: ISSN-0010-0277
Language: English Document Type: JOURNAL ARTICLES (080); VIEWPOINTS (120); REPORTS (143)
Journal Announcement: CIJDEC2000
Argues that there is a social-cognitive and a social-perceptual component to a theory of mind. Presents as evidence three studies comparing children with Williams syndrome to children with Prader-Willi syndrome, and to children with nonspecific mental retardation. The findings suggest that only social-perceptual components of theory of mind, which are linked to distinct neurobiological substrates, are spared.
Descriptors: *Children; *Cognitive Development; Comparative Analysis; Disabilities; *Mental Retardation; Neurological Impairments; Social Cognition; *Theories;
Identifiers: Prader Willi Syndrome; *Theory of Mind; Williams Syndrome

EJ617940 EC626223
Williams Syndrome and Happiness.
Levine, Karen; Wharton, Robert
American Journal on Mental Retardation; v105 n5 p363-71 Sep 2000
ISSN: ISSN-0895-8017
Language: English
Document Type: JOURNAL ARTICLES (080); VIEWPOINTS (120)
Journal Announcement: CIJJUN2001
Discussion of Williams syndrome, a genetic disorder with a variety of medical and developmental features, focuses on frequent outward expression of happiness. Analysis of the unique expression of happiness in individuals with Williams syndrome is followed by discussion of this happiness in the context of other dimensions of the syndrome, especially anxiety. The role of genetics in emotions is also addressed.
Descriptors: Anxiety; *Congenital Impairments; Emotional Development; *Genetics; Happiness; *Psychological Characteristics; *Special Health Problems;
Identifiers: *Williams Syndrome

EJ485021 EC608704
Four Case Histories and a Literature Review of Williams Syndrome and Autistic Behavior.
Gillberg, Christopher; Rasmussen, Peder
Journal of Autism and Developmental Disorders, v24 n3 p381-93 Jun 1994
ISSN: 0162-3257
Language: English
Journal Announcement: CIJOCT94
This paper summarizes the case histories of four young children with concurrent autistic disorder and Williams syndrome. Williams syndrome comprises a peculiar facial appearance, learning disorder, and often hypercalcemia, mild microcephaly, large blood vessel stenosis, and a specific behavioral phenotype. Literature on Williams syndrome is reviewed, focusing on prevalence, biochemical abnormalities, chromosome abnormalities, and brain morphology. Descriptors: *Autism; *Behavior Patterns; Case Studies; Incidence; *Multiple Disabilities; *Symptoms (Individual Disorders); Young Children
Identifiers: *Williams Syndrome

EJ545904 EC615657
Brief Report: Response to Methylphenidate in Two Children with Williams Syndrome.
Power, Thomas J.; And Others
Journal of Autism and Developmental Disorders, v27 n1 p79-87 Feb 1997
ISSN: 0162-3257
Language: English
Journal Announcement: CIJNOV97
This study explored the use of methylphenidate with two boys (ages 7 and 8 years) with Williams syndrome, a genetic disorder frequently accompanied by symptoms associated with Attention Deficit Hyperactivity Disorder (ADHD). Both boys performed optimally on 10 mg of methylphenidate administered twice daily, although one displayed increased passivity at home. Contextual effects are also discussed.
Descriptors: *Attention Deficit Disorders; *Behavior Problems; Children; *Drug Therapy; *Outcomes of Treatment; Pharmacology
Identifiers: *Methylphenidate; *Williams Syndrome

ED411652 EC305844
Williams Syndrome: A Family's Journey
Sorrell, Adrian L.
U.S.; Texas
NOTE Paper presented at the Annual International Conference on Mental Retardation and Developmental Disabilities (5th, Austin, TX, October 11, 1996).
PTYP 055; 150
This conference paper describes Williams syndrome, a rare genetic disorder that results in mental retardation. An overview of this condition is presented from the perspective of a family who traveled through the many stages of the disability from infancy to young adulthood. The etiology and characteristics of the disability are discussed, including distinctive facial features, delayed motor development, and speech patterns. The paper also addresses the educational implications of the disability. The behavior problems of children with Williams syndrome are identified, including their short attention spans, difficulty in modulating emotions, anxiety around unexpected changes in routine, heightened sensitivity to sounds, preseverating on favorite conversational topics, rocking, and difficulty building friendships. Testing, placement, and academic difficulties are also discussed along with suggestions on how to make appropriate accommodations. The paper closes with a description of the educational career of a child with Williams syndrome. A list of resource organizations is provided and information summary sheets are attached for overhead display.
Descriptors: *Behavior Problems; Disability Identification; * Elementary Secondary Education; Etiology; *Interpersonal Competence; *Mental Retardation; Peer Relationship; *Student Characteristics; Student Placement; Teaching Methods;
Identifiers: *Academic Accommodations (Disabilities); Testing Accommodations (Disabilities); *Williams Syndrome

EJ546586 SO528845
Special Learners with Special Abilities.
Stambaugh, Laura
Music Educators Journal, v83 n3 p19-23 Nov 1996
ISSN: 0027-4321
Language: English
Journal Announcement: CIJNOV97
Target Audience: Teachers; Practitioners
Profiles a summer music and arts program specifically designed for children and adults with Williams Syndrome. Williams Syndrome is a chromosomal aberration, usually resulting in mild or moderate retardation often accompanied by an acute sensitivity to sound and perfect pitch. The summer program focused on music and performing arts. Descriptors: Adult Education; Applied Music; Congenital Impairments; *Disabilities; Elementary Secondary Education; *Mental Retardation; Music Activities; *Music Education; *Music Therapy; *Resident Camp Programs; *Special Education; Summer Programs; Therapeutic Environment; Therapeutic Recreation
Identifiers: Massachusetts; *Williams Syndrome

EJ399068 EC221160
Language and Cognition in Two Children with Williams Syndrome.
Thal, Donna; And Others
Journal of Speech and Hearing Research, v32 n3 p489-500 Sep 1989
Language: English
Journal Announcement: CIJAPR90
Target Audience: Researchers
The study examined two female children (ages two and five) with Williams Syndrome (characterized by moderate to severe mental retardation with verbal intelligence quotients significantly higher than performance intelligence quotients). Comparison with both normal and delayed children indicated an unusual cognitive profile.
Descriptors: Case Studies; *Cognitive Development; Females; *Language Acquisition; *Mental Retardation; *Verbal Ability; Young Children
Identifiers: *Williams Syndrome

For more information, contact:
Williams Syndrome Association
PO Box 297
Clawson, MI 48017-0279

CAWS: Canadian Association for Williams Syndrome

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