CD-ROM Today 1996 January/CD-ROM Today 1996 January.iso/dp/0434/04343.txt

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$Unique_ID{BRK04343}
$Pretitle{}
$Title{XYY Syndrome}
$Subject{XYY Syndrome 47,XYY Syndrome 47,XYY Karyotype Polysomy Y Diplo-Y
Syndrome YY Syndrome XYY Chromosome Pattern Klinefelter Syndrome Sotos
Syndrome Marfan Syndrome Antisocial Personality Disorder }
$Volume{}
$Log{}

Copyright (C) 1990, 1992 National Organization for Rare Disorders, Inc.

812:
XYY Syndrome

** IMPORTANT **
It is possible that the main title of the article (XYY Syndrome) is not
the name you expected.  Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     47,XYY Syndrome
     47,XYY Karyotype
     Polysomy Y
     Diplo-Y Syndrome
     YY Syndrome
     XYY Chromosome Pattern

Information on the following disorders can be found in the Related
Disorders section of this report:

     Klinefelter Syndrome
     Sotos Syndrome
     Marfan Syndrome
     Antisocial Personality Disorder

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.

XYY Syndrome is a rare chromosomal disorder which affects males.  It is
caused by the presence of an extra Y chromosome.  Healthy males have one X
and one Y chromosome.  Men with XYY Syndrome are usually very tall and thin.
Many have severe acne during adolescence.  Other symptoms may include lower
than average intelligence and antisocial or behavioral problems.

Symptoms

Characteristics of XYY Syndrome are often subtle and do not indicate a
serious chromosomal disorder.  Thus, males with this condition are often
undiagnosed or misdiagnosed.  Major symptoms may include tall or very tall
height (usually becoming apparent after the age of 5 or 6), and severe cystic
acne during adolescence.  (For more information on cystic acne, choose "acne"
as your search term in the Rare Disease Database).

Lower than average intelligence, and/or behavioral problems (such as an
explosive temper, aggressive or defiant actions, or sometimes antisocial
behavior) are other symptoms.  Some individuals with this disorder may have
language difficulties or psychosexual problems.  XYY Syndrome is often
undiagnosed until tests for other medical reasons are performed.  Other than
being exceptionally tall and/or having behavioral problems, in many instances
these boys or men appear normal.

Physical characteristics of XYY Syndrome may include an unusually long
head with a slightly protrusive forehead, long hands and feet, long ears,
mild indentation in the lower area of the breastbone (pectus excavatum),
and/or large teeth.  Poor chest and shoulder muscle development (pectoral and
shoulder girdle musculature) is also common.  Even though males with this
syndrome are large, they tend to be weak and uncoordinated.  Some may have a
fine intentional tremor (e.g. their hands may shake when they try to drink a
glass of water).

Occasionally, a bony formation across the joints in the two bones of the
forearm resulting in stiffening of the affected joints (radioulnar
synostosis) may occur.  Other occasional symptoms are undescended testicles
(cryptorchidism), a small penis, or an opening located on the underside of
the penis (hypospadias).

For a long time it was believed that XYY Syndrome individuals had
aggressive tendencies often associated with criminal behavior due to the
extra Y chromosome.  Epidemiological studies suggest that one out of every 35
institutionalized male juvenile delinquents has XYY Syndrome.  However, it is
now believed by some researchers that this behavior is not due to the extra Y
chromosome, but rather to the lower than average intelligence and education
levels of these men.  More research is needed to understand the role of this
chromosomal abnormality on behavior.

Causes

XYY Syndrome is a genetic disorder caused by the presence of an extra Y
chromosome.  Normal males have 46 chromosomes including one X and one Y
chromosome.  Men with XYY Syndrome have 47 chromosomes, two of which are Y
chromosomes.  Why the extra Y chromosome occurs is not known.  In very rare
instances, the syndrome has been passed from father to son, but in most cases
heredity cannot be established.

Affected Population

XYY Syndrome is a chromosomal disorder present at birth that affects only
males.  It is estimated to occur in approximately 1 of 1000 live births.

Related Disorders

Symptoms of the following disorders can be similar to those of XYY Syndrome.
Comparisons may be useful for a differential diagnosis:

Klinefelter Syndrome is characterized by the presence of one or more
extra x-chromosomes.  It affects only males.  Individuals with this syndrome
tend to be tall and slim in childhood.  A striking lack of muscular
development, and a small penis and testicles may also occur.  Lower than
average intelligence, language difficulties, intention tremor, and behavioral
problems may be other symptoms.  (For more information on this disorder,
choose "Klinefelter" as your search term in the Rare Disease Database).

Sotos Syndrome is a rare, hereditary disorder characterized by excessive
growth during the first 4 to 5 years of life.  Other symptoms may include an
unusual aggressiveness or irritability, clumsiness and an awkward way of
walking.  People with this disorder have abnormal patterns of the ridges on
the skin of the fingers, palms, toes and soles (dermatoglyphics).  Patients
have a disproportionately large and long head, with a slightly protrusive
forehead, large hands and feet.  Mild mental retardation may also occur.
(For more information on this disorder, choose "Soto" as your search term in
the Rare Disease Database).

Marfan Syndrome is an inherited disorder of the connective tissues.
People with this disorder tend to be unusually tall and thin with large hands
and feet.  The face may appear long, and the breastbone may be protruding or
indented.  They may walk with an irregular or unsteady gait.  People with
Marfan Syndrome have normal intelligence, and they do not have any behavioral
symptoms.  (For more information on this disorder, choose "Marfan" as your
search term in the Rare Disease Database).

Antisocial Personality Disorder is a mental illness characterized in
early childhood by behavior such as lying, stealing, fighting, truancy and
resisting authority.  During adolescence, there may be excessive drinking,
use of illicit drugs and aggressive sexual behavior.  The behavioral
difficulties usually last throughout adult life.  In many cases disrespect
for authority leads to problems with the law.  (For more information on this
disorder, choose "Antisocial Personality Disorder" as your search term in the
Rare Disease Database).

Therapies:  Standard

Treatment of XYY Syndrome is symptomatic and supportive.  Counseling for
behavioral or sexual problems may be of benefit.  Treatment of acne may help
the patient's self-image.

Therapies:  Investigational

Anyone who had a prenatal diagnosis of XYY Syndrome and is between the ages
of five and twenty may wish to participate in a study being conducted to
determine mental and behavioral outcomes connected with this syndrome.
Interested persons may wish to contact Dr. John M. Graham of the UCLA School
of Medicine, c/o KS and Associates, P.O. Box 119, Roseville, CA, 95661-0119.

This disease entry is based upon medical information available through
September 1992.  Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate.  Please check with the agencies listed in the Resources section for
the most current information about this disorder.

Resources

For more information on XYY Syndrome, please contact:

     National Organization for Rare Disorders
     P.O. Box 8923
     New Fairfield, CT 06812-1783
     [phone]

     National Mental Health Association
     1021 Prince St.
     Alexandria, VA  22314
     [phone]

     National Alliance for the Mentally Ill
     1901 N. Fort Meyer Dr., Suite 500
     Arlington, VA  22209
     [phone]

     National Mental Health Consumer Self-Help Clearinghouse
     311 S. Juniper St., Rm. 902
     Philadelphia, PA  19107
     [phone]

     NIH/National Institute of Mental Health (NIMH)
     9000 Rockville Pike
     Bethesda, MD 20205
     [phone] or [phone]
     [phone] (24 hrs.)

     International Tremor Foundation
     360 W. Superior St.
     Chicago, IL  60610
     [phone]

For genetic information and genetic counseling referrals, please contact:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY  10605
     [phone]

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     [phone]

References

CECIL TEXTBOOK OF MEDICINE, 18th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1988.  Pp. 167.

INTERNAL MEDICINE, 2nd Ed.:  Jay H. Stein, ed.-in-chief; Little, Brown
and Co., 1987.  Pp. 1989.

THE MERCK MANUAL, Volume 1, 15th Ed.:  Robert Berkow, M.D., ed.-in-chief;
Merck, Sharp, and Dohme Laboratories, 1987.  Pp. 2150.

SMITH'S RECOGNIZABLE PATTERNS OF HUMAN MALFORMATION, 4th Ed.:  Kenneth
Lyons Jones, M.D.; W.B. Saunders Co., 1988.  Pp. 64-66.

EFFECTS OF THE Y CHROMOSOME ON QUANTITATIVE GROWTH:  AN ANTHROPOMETRIC
STUDY OF 47,XYY MALES.  J. Varrela and L. Alvesalo; Am J Phys Anthropol; (Oct
1985; issue 68 (2)).  Pp. 239-245.

SEX CHROMOSOME ANOMALIES, HORMONES, AND SEXUALITY.  R. C. Schiavi, et
al.; Arch Gen Psychiatry; (Jan 1988; issue 45 (1)).  Pp. 19-24.

SEX CHROMOSOME VARIATIONS IN SCHOOL-AGE CHILDREN.  F. L. Cohen and J.D.
Durham; J Sch Health; (Mar 1985; issue 55 (3)).  Pp. 99-102.

SPERM CHROMOSOME COMPLEMENTS IN A 47,XYY MAN.  J. Benet and R. H. Martin;
Hum Genet; (Apr 1988; issue 78 (4)).  Pp. 313-315.