$Unique_ID{BRK04234}
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$Title{Stein-Leventhal Syndrome}
$Subject{Stein-Leventhal Syndrome Polycystic Bilateral Ovarian Syndrome
Bilateral Polycystic Ovarian Syndrome Ovarian Hyperthecosis Sclerocystic
Ovarian Disease }
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Copyright (C) 1986, 1989, 1991 National Organization for Rare Disorders,
Inc.

111:
Stein-Leventhal Syndrome

** IMPORTANT **
It is possible that the main title of the article (Stein-Leventhal
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

     Polycystic Bilateral Ovarian Syndrome
     Bilateral Polycystic Ovarian Syndrome
     Ovarian Hyperthecosis
     Sclerocystic Ovarian Disease

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.


Stein-Leventhal Syndrome is a reproductive disorder affecting young
women.  It is characterized by absent or abnormal menstruation, sterility,
mild signs of virilization, and sometimes obesity.  The causes of the
syndrome are not understood, but may involve faulty production of
reproductive hormones.  Therapy is often effective, but recurrences are
common.

Symptoms

The first symptoms of Stein-Leventhal syndrome usually appear shortly after
puberty and before age twenty, after menstruation has been normal or
irregular for some time.  Menstrual flow becomes irregular and gradually
decreases over several months, until menstruation ceases.  Sometimes, profuse
menstrual flow interrupts longer intervals of amenorrhea.  Menstrual pain
occurs in about 20% of the patients.

Many patients consult a physician because of infertility or the
appearance of increased facial hair.  Other signs of virilization include
voice changes and increased body hair with a "male" distribution, such as on
the chest.  Obesity is a frequently associated problem.

Cysts are found directly under the surface of the ovaries.  Other
pathological changes also occur.  The uterus is often small and
underdeveloped.

Stein-Leventhal syndrome should be treated even when pregnancy is not
desired because erratic, or breakthrough bleeding can be a problem, and
because changes in the endometrium, the tissue lining the uterus that changes
regularly in the normal menstrual cycle, can lead to precancerous or
cancerous conditions.

Causes

The causes of Stein-Leventhal syndrome may vary from case to case, and are
usually poorly understood.  Generally, the abnormalities are thought to occur
in hypothalamic, pituitary, or ovarian hormone production.  Occasionally, a
tumor of the adrenal gland produces excessive amounts of androgenic hormones.
In all cases, the ovary fails to ovulate, and through a feed back mechanism,
becomes enlarged and full of cysts.

Affected Population

Young women are affected by Stein-Leventhal Syndrome.

Therapies:  Standard

If a patient afflicted with Stein-Leventhal Syndrome wants to become
pregnant, ovulation and normal menstruation follows administration of
clomiphene in many cases; pregnancy occurs in about half of these patients.
If clomiphene is ineffective, gonadotropins may be tried.  Should both fail,
ovarian wedge resection, or removal of the cystic portions of the ovaries,
can be performed.  However, symptoms often recur.

If pregnancy is not desired, the ovary can be fully suppressed with low
dosage birth control pills or long acting progestins such as
medroxyprogesterone.

The investigational drug Flutamide is being tested in Stein-Leventhal
Syndrome as a treatment for the control of hair growth.  For further
information physicians may contact:

     Dr. David Ehrmann
     Department of Medicine
     University of Chicago Medical Center
     5841 Maryland Ave., Box 435
     (312) 702-9653

Therapies:  Investigational

This disease entry is based upon medical information available through
November 1991.  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 Stein-Leventhal Syndrome, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     NIH/National Institute of Child Health and Human Development (NICHHD)
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-5133

References

THE MERCK MANUAL 15th ed:  R. Berkow, et al: eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  Pp. 1056, 1695.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  Pp. 1287.