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Fragile X Syndrome (updated March 2003)

What information do you have on Fragile X Syndrome?

The following information is from the FRAXA Research Foundation.

Symptoms of fragile X syndrome include:

  • Mental impairment, ranging from learning disabilities to mental retardation
  • Attention deficit and hyperactivity
  • Anxiety and unstable mood
  • Autistic-like behaviors
  • Long face, large ears, flat feet, and
  • Hyperextensible joints, especially fingers
Boys are typically more severely affected than girls. While most boys have mental retardation, only one-third to one-half of girls have significant intellectual impairment; the rest have either normal IQ or learning disabilities. Emotional and behavioral problems are common in both sexes.

Fragile X syndrome is the single most common inherited cause of mental impairment. Current estimates of its prevalence vary, but some experts believe that fragile X affects at least 1 in 1000 males and females of all races and ethnic groups. More conservative estimates put the frequency at 1 in 1500 males and 1 in 2500 females (Warren, Jama, Feb. 16, 1994 - Vol.271, No.7, p.536). The discrepancy in these numbers is due to the fact that large-scale population studies of the incidence of fragile X have not yet been done. In either case, fragile X is one of the most common genetic diseases in humans. It is estimated that 80-90% of people with fragile X are not yet correctly diagnosed. In 1991, scientists discovered the gene (called FMR1) that causes fragile X. In individuals who have fragile X syndrome, a defect in FMR1 (a full mutation) shuts the gene down. Like a defective factory, the FMR1 gene cannot manufacture the protein that it normally makes. Other individuals are carriers: they have a small defect in the FMR1 gene (called a premutation) but do not show symptoms of fragile X.

Fragile X is inherited. Carrier men (transmitting males) pass the premutation to all their daughters but none of their sons. Each child of a carrier woman has a 50% chance of inheriting the gene. The fragile X premutation can be passed silently down through generations in a family before a child is affected by the syndrome.

Because the symptoms of fragile X can be quite subtle, especially in young children, and because fragile X is so frequent in the general populations, many medical specialists recommend that testing for fragile X be considered for any individual with otherwise unexplained developmental delay or mental retardation. Most major medical centers in the United States now offer the DNA test for fragile X. This test typically costs $200-$300.

There is currently no cure for fragile X syndrome, although appropriate education and medications can help maximize the potential of each child. However, most boys and many girls remain significantly affected throughout their lives.

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 primary focus of the ERIC system 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

fragile X syndrome

ED406787 EC305488
Fragile X Syndrome in Males: Diagnostic, Behavioral, and Educational Implications.
Bellinger, Diane; And Others
Lynchburg Coll., VA 1995
27p.
EDRS Price - MF01/PC02 Plus Postage.
Language: English
Document Type: REVIEW LITERATURE (070)
Geographic Source: U.S.; Virginia
Journal Announcement: RIESEP97
This paper reviews the research on fragile X syndrome, the second most common cause of mental retardation related to chromosomal anomaly. It notes that far more males than females are affected by the fragile X syndrome, which typically results in craniofacial changes, delays in growth and development, speech/language difficulties, and cognitive deficits including mental retardation or learning disabilities. Topics reviewed include: diagnostic methods (direct DNA analysis is now preferred to cytogenetic analysis or DNA linkage studies); inheritance patterns (which explain the predominance of males); physical and cognitive characteristics; medical conditions associated with the condition (seizure disorders, motor coordination deficits); behavioral characteristics (hyperactivity, gaze avoidance); and speech/language characteristics (perseveration, cluttering, and echolalia). Other topics discussed are a possible relationship between autism and fragile X syndrome, whether language problems characteristic of the syndrome are due to qualitative or quantitative differences in language development, and the common presence of hypersensitivity. Interventions reviewed are organized into medical (especially drug therapy), and educational, which include classroom modifications, training in self-management skills, curricular modifications, and sensory integration therapy.
Descriptors: Autism; Behavior Patterns; Clinical Diagnosis; DNA; Drug Therapy; Educational Needs; Failure to Thrive; *Genetics; Heredity; *Intervention; Language Acquisition; Language Impairments; Learning Disabilities; Males; Medical Services; *Mental Retardation; *Multiple Disabilities; Sensory Integration; Special Health Problems; *Symptoms (Individual Disorders)
Identifiers: *Fragile X Syndrome

ED376631 EC303458
Male-Female Characteristics of Fragile X Syndrome.
Caron, Jackie
Jan 1994
21p.
EDRS Price - MF01/PC01 Plus Postage.
Language: English
Document Type: REVIEW LITERATURE (070); PROJECT DESCRIPTION (141)
Geographic Source: U.S.; New Mexico
Journal Announcement: RIEAPR95
Fragile X syndrome is the most common cause of mental retardation next to Down syndrome. The syndrome is more prevalent in males because they only have one X chromosome, where the gene for fragile X is carried, while women have two X chromosomes and the normal gene can compensate for the affected chromosome. Certain physical features are associated with the syndrome, such as elongated faces and hyperextensible finger joints. Children with fragile X have IQs that decline as they become adults. Language problems affect all males with fragile X, even those that have a normal IQ. Many prepubertal males may have borderline IQs, learning disabilities, attention deficits, and hyperactivity. Primary weaknesses include arithmetic, short-term memory, and signs of autistic behavior that lasts into adulthood. Women with fragile X show milder symptoms than men, both in physical and cognitive problems. One of the main treatments for fragile X is folic acid, along with special education, language therapy, occupational therapy, and psychotherapy. Genetic counseling is very important for anyone who is aware of having or being a carrier of the fragile X syndrome.
Descriptors: Adults; Autism; Children; *Cognitive Development; *Congenital Impairments; Elementary Secondary Education; Females; Genetics; Heredity; Incidence; Intervention; Males; *Mental Retardation; *Sex Differences; *Symptoms (Individual Disorders)
Identifiers: *Fragile X Syndrome

ED458780 EC308733
Fragile X Syndrome: A Guide for Teachers.
Saunders, Suzanne
2001
114p.
ISBN: ISBN-1-85346-536-4
Available from: Taylor & Francis, Inc., 7625 Empire Dr., Florence, KY 41042 ($23.95). Tel: 800-634-7064 (Toll Free); Fax: 800-248-4724 (Toll Free); Web site: http://www.taylorandfrancis.com.
EDRS Price: Document Not Available from EDRS.
Language: English
Document Type: BOOKS (010); GUIDES (055)
Geographic Source: United Kingdom; England
Journal Announcement: RIEAPR2002
This book is intended to give educators an overview of Fragile X syndrome, including how it affects individuals, usually males, and how teachers can use strategies and methods suited to their specific needs. Chapter 1 briefly describes those medical aspects of Fragile X that teachers may find helpful. Chapter 2 looks at typical characteristics of children with Fragile X, including physical, speech and language, behavioral, and intellectual and cognitive characteristics. Chapter 3 looks at the classroom environment and offers modification suggestions to help children with Fragile X function best. Chapter 4 looks specifically at the cognitive profile of children with Fragile X and identifies their strengths and weaknesses in learning. It also suggests ways that teachers can adapt teaching methods and strategies most likely to be effective. Chapter 5 considers particular behavioral and social characteristics associated with Fragile X and those also common to children with attention deficit hyperactivity disorder and to children on the autistic continuum. Various methods to manage and modify aspects of the child's behavior such as drug therapy are discussed. Finally, Chapter 6 focuses on the families of children with Fragile X and offers the perspective of different family members.
Descriptors: Behavior Change; Behavior Problems; *Congenital Impairments; *Developmental Disabilities; Elementary Secondary Education; *Intervention; Males; Mental Retardation; Parent Attitudes; Special Health Problems; Student Characteristics; Student Needs
Identifiers: *Fragile X Syndrome

ED460556 EC308774
Elementary School and Boys with Fragile X Syndrome. Final Report.
Bailey, Don; Hatton, Deborah
2001
39p.
EDRS Price: MF01/PC02 Plus Postage.
Language: English
Document Type: REPORTS (143); TESTS, EVALUATION INSTRUMENTS (160)
Geographic Source: U.S.; North Carolina
Journal Announcement: RIEJUN2002
This final report reviews the second phase of a life-span study of boys with fragile X syndrome (FSX), the most common known inherited cause of mental retardation. Males with the syndrome are more severely affected than females and in males, delays are usually evident in all the developmental domains, although cognitive and communication skills are likely to be most affected. The project studied 61 elementary school aged boys and their families. Approximately 25% of the boys were found to also meet diagnostic criteria for autism. It was noted that when autistic behavior and FXS co-occur, the effect on development appears to be additive. The heart rates of the boys with FXS were significantly higher than of typically developing children, and their visual-motor skills were less than half than would be typically expected. Achievement test scores showed wide variability. The children with FXS were found to be hypersensitive to their environment and about 43% had clinically significant behavior problems in the classroom. By third grade, 85% of the children were in self-contained, rather than inclusive classrooms. Teachers reported that 55% of the children had at least one friend at school and parents reported that 72% had at least one friend in or out of school. In most cases, the friend was another child with a disability. In addition to these findings, strategies to teach reading skills are discussed in the report. Data were collected on families' needs and supports, but have not yet analyzed. Budget information, a list of presentations, and a list of published articles related to the study are included.
Descriptors: *Autism; Behavior Patterns; Children; Cognitive Ability; Cognitive Development; *Developmental Disabilities; DNA; Elementary Education; Emotional Disturbances; Friendship; Genetics; Heredity; Intelligence Differences; Longitudinal Studies; *Males; *Mental Retardation; Sensory Integration; Social Development; *Symptoms (Individual Disorders)
Identifiers: *Fragile X Syndrome

ED449607 EC308213
Longitudinal Changes in Cognitive Ability and Adaptive Behavior in Children and Adolescents with the Fragile X Mutation or Autism.
Simensen, R. J.; Fisch, G. S.; Schroer, R. J.
2000
20p.
EDRS Price: MF01/PC01 Plus Postage.
Language: English
Document Type: REPORTS (143); SPEECHES, COFERENCE PAPERS(150)
Geographic Source: U.S.; South Carolina
Journal Announcement: RIEJUL2001
This report discusses the outcomes of a study that examined 216 comparably aged children and adolescents (ages 2-18) with fragile X or autism to determine whether longitudinal change in cognitive ability and adaptive behavior was similar in the two groups. Results found decreases in Intelligence Quotient scores in young children with fragile X as well as those with autism. Older children and adolescents with autism exhibited stable test-retest scores, while older children with fragile X continued to show decreases. Comparable declines in adaptive behavior were observed in both groups, at all ages, and across all adaptive behavior domains.
Descriptors: Adolescents; Age Differences; *Autism; Children; *Cognitive Ability; *Cognitive Development; *Intelligence Differences; Longitudinal Studies; *Mental Retardation; *Symptoms (Individual Disorders)
Identifiers: *Fragile X Syndrome

EJ627904 EC627462
Conversational Characteristics of Children with Fragile X Syndrome: Repetitive Speech.
Belser, Richard C.; Sudhalter, Vicki
2001
American Journal on Mental Retardation; v106 n1 p28-38 Jan 2001
ISSN: ISSN-0895-8017
Language: English
Document Type: JOURNAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJDEC2001
Comparison of the production of repetitive speech during conversations in 30 people with either fragile X syndrome, autistic disorder, or mental retardation not caused by fragile X found repetitive speech more prevalent among those with fragile X. Results support the hypothesis that such speech dysfluency reflects the effects of physiological arousal caused by hypersensitivity to social and sensory stimuli.
Descriptors: Autism; Children; *Congenital Impairments; *Etiology; *Genetics; Incidence; *Mental Retardation; *Speech Impairments.
Identifiers: *Fragile X Syndrome; *Repetition (Language)

EJ629473 EC627835
Identifying Infants and Toddlers with Fragile X Syndrome: Issues and Recommendations.
Bailey, Donald B., Jr.; Roberts, Jane E.; Mirrett, Penny; Hatton, Deborah D.
2001
Infants and Young Children; v14 n1 p24-33 Jul 2001
ISSN: ISSN-0896-3746
Language: English
Document Type: GUIDES (055); JOURNAL ARTICLES (080)
Journal Announcement: CIJJAN2002
This article discusses the difficulties of early identification of Fragile X syndrome (FXS), provides an overview and analysis of existing screening checklists, and recommends a set of characteristics to enhance the identification process of infants and toddlers with FXS. Indicators address family history, behavior, social/communication, oral-motor/feeding, and physical characteristics.
Descriptors: *Check Lists; *Clinical Diagnosis; *Early Identification; Evaluation Methods; Infants; *Mental Retardation; *Screening Tests; *Symptoms (Individual Disorders); Toddlers
Identifiers: *Fragile X Syndrome

EJ627903 EC627461
Variability in FMRP and Early Development in Males with Fragile X Syndrome.
Bailey, Donald B., Jr.; Hatton, Deborah D.; Tassone, Flora; Skinner, Martie; Taylor, Annette K.
American Journal on Mental Retardation; v106 n1 p16-27 Jan 2001
ISSN: ISSN-0895-8017
Language: English
Document Type: JOURNAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJDEC2001
This study examined whether the variability in development in fragile X syndrome is related to FMR protein deficiency in 53 young males with various degrees of the syndrome. FMR protein expression accounted for a small but significant amount of variance in level, but not rate, of development. Findings support the hypothesized relationship but suggest other factors also play a role.
Descriptors: Biochemistry; *Congenital Impairments; *Etiology; *Genetics; Males; *Mental Retardation; Young Children Identifiers: *Fragile X Syndrome; Proteins

EJ526408 PS525095
Biomedical Advances in Developmental Psychology: The Case of Fragile X Syndrome.
Hagerman, Randi J.
Developmental Psychology, v32 n3 p416-24 May 1996
This article is part of a special section: Biomedicine and Developmental Psychology: New Areas of Common Ground.
ISSN: 0012-1649
Language: English
Document Type: RESEARCH REPORT (143); JOURNAL ARTICLE (080)
Journal Announcement: CIJNOV96
Discusses the Human Genome Project and the identification of Fragile X Syndrome, the most common inherited cause of mental retardation. Fragile X Syndrome is caused by an abnormal gene on the bottom of the X chromosome. Examined the phenotype of Fragile X Syndrome in males and females and the spectrum of learning difficulties caused by the mutation.
Descriptors: *Biomedicine; Developmental Psychology; *Genetics; Learning Disabilities; *Medical Research; Mental Retardation; Sex Differences
Identifiers: *Fragile X Syndrome; Human Genome Project

ED364033 EC302603
International Fragile X Conference Proceedings (3rd, Snowmass/Aspen, Colorado, 1992).
Hagerman, Randi Jenssen, Ed.; McKenzie, Pamela, Ed.
National Fragile X Foundation, Denver, CO. 1992
399p.
ISBN: 0-915667-22-3
Available From: National Fragile X Foundation, 1441 York Street, Suite 215, Denver, CO 80206 ($26.95; 10 or more $20 each plus shipping and handling).
EDRS Price - MF01 Plus Postage. PC Not Available from EDRS.
Language: English
Document Type: CONFERENCE PROCEEDINGS (021)
Geographic Source: U.S.; Colorado
Journal Announcement: RIEAPR94
This proceedings document presents the texts or summaries of 52 papers given at a 1992 conference on Fragile X syndrome. Preliminary information includes names and institutional affiliations of conference faculty, information about the National Fragile X Foundation, awards presented at the conference, and a list of resource centers (by state). Papers are organized into the following four categories: (1) physical, cognitive, and behavioral phenotype and medical follow up (e.g., medical management, physical features, seizures, oculovisual dysfunction, learning disability, socioemotional characteristics of adult mentally retarded men with Fragile X syndrome, behavioral assessments, and psychosis); (2) educational and behavioral interventions (e.g., the Fragile X Educational Assistance Program, combined occupational and speech therapy, assistive technology, psychotherapy, vocational training, and facilitating a parent/family support group; (3) cytogenetic and molecular diagnostic testing (e.g., molecular genetics, the genetic lesion causing the disorder, genetic counseling, prenatal diagnosis, cytogenetics, prevalence of chromosomal abnormalities, and carrier testing of persons with suggestive family histories); and (4) social concerns (screening, economic impact of the Fragile X syndrome in Colorado, and ethical issues in the provision of genetic services). Papers include references.
Descriptors: *Clinical Diagnosis; *Congenital Impairments; Diagnostic Tests; *Educational Methods; Educational Needs; Family Counseling; *Genetics; Individual Characteristics; Intervention; Learning Disabilities; Medical Evaluation; *Mental Retardation; Multiple Disabilities; Sex Differences; Special Health Problems; *Symptoms (Individual Disorders); Therapy
Identifiers: *Fragile X Syndrome

EJ444471 EC602943
Fragile X Syndrome: An Educator's Role in Identification, Prevention, and Intervention.
Santos, Karen E.
Remedial and Special Education (RASE), v13 n2 p32-39 Mar-Apr 1992
ISSN: 0741-9325
Language: English
Document Type: REVIEW LITERATURE (070); JOURNAL ARTICLE (080)
Journal Announcement: CIJSEP92
This review highlights the physical, intellectual, academic, behavioral, and communication characteristics associated with fragile X syndrome, the most common inherited form of mental retardation. Educationally relevant problems linked to fragile X are noted, including learning disabilities, attention deficit disorders, speech and language deficits, autism, and behavior disorders.
Descriptors: Attention Deficit Disorders; Autism; Behavior Disorders; Elementary Secondary Education; *Genetics; *Incidence; Language Handicaps; Learning Disabilities; *Mental Retardation; Speech Handicaps; *Student Characteristics; *Symptoms (Individual Disorders)
Identifiers: *Fragile X Syndrome

AUTH Schopmeyer, Betty B., Ed.; Lowe, Fonda, Ed.
TITL The Fragile X Child.
REP ISBN-1-879105-83-7
AVAL Singular Publishing Group, Inc., 4284 41st St., San Diego, CA 92105-1197 ($39.95).
PTYP 010; 070
AUD Support Staff
DESC Congenital Impairments; Elementary Education; Genetics; Child Health; Occupational Therapy; Speech Therapy; Case Studies; Teaching Methods; Disability Identification; Clinical Diagnosis
IDEN Fragile X Syndrome
ABST This book is written to assist clinicians and therapists in recognizing, assessing, and planning effective treatment for children with fragile X syndrome. The first section provides an overview of background information necessary to understand the syndrome, including "The Genetics of Fragile X Syndrome" (Susan Black) and "Medical Aspects of the Fragile X Syndrome" (Randi Hagerman). Part II examines the characteristics of children with fragile X in several domains, and includes the following chapters: "Occupational Therapy for Children with Fragile X Syndrome" (Sharon Kaye); "Speech and Language Therapy with the Fragile X Child" (Fonda Lowe); and "Combined Treatment" (Lowe, Schopmeyer, and Kaye). A final section consists of a single chapter by Betty Schopmeyer and Sharon Kaye applying these techniques in a case study. An appendix lists people and organizations to contact for further information.
 

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