$Unique_ID{BRK03748}
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$Title{Forbes-Albright Syndrome}
$Subject{Forbes-Albright Syndrome Nonpuerperal Amenorrhea-Galactorrhea
Nonpuerperal Galactorrhea Galactorrhea-Amenorrhea Syndrome}
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Copyright (C) 1986, 1989 National Organization for Rare Disorders, Inc.

153:
Forbes-Albright Syndrome

** IMPORTANT **
It is possible that the main title of the article (Forbes-Albright
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

     Nonpuerperal Amenorrhea-Galactorrhea
     Nonpuerperal Galactorrhea
     Galactorrhea-Amenorrhea Syndrome

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.


Forbes-Albright Syndrome belongs to a group of disorders characterized by
abnormal lactation (milk production) and, in women, an absence of menstrual
periods.  Hormone secreting tumors in the pituitary region cause almost all
cases of the syndrome.  Forbes-Albright Syndrome is not correlated with
pregnancy.  It responds to pharmacological treatment for variable periods of
time.

Symptoms

Onset of Forbes-Albright Syndrome usually occurs during the patient's
twenties or thirties.  In women, the symptoms consist of galactorrhea
(abnormal secretion of a milky substance from the nipples) and an absence of
menstrual periods (amenorrhea).  The breasts and nipples are of normal size
and appearance, but the secondary sexual characteristics, such as hair
distribution, may change somewhat.  Some patients may become obese, and the
skin may become unusually oily.

In men, the breasts may enlarge and begin to secrete milk.

Laboratory tests reveal elevated levels of prolactin, the hormone
responsible for lactation after childbirth.  In addition, they indicate low
levels of gonadotropins, hormones such as Follicle Stimulating Hormone (FSH),
that regulate the monthly ovulatory cycle.

Causes

Forbes-Albright Syndrome is caused by hormone secreting tumors in the region
of the pituitary gland, and sometimes the hypothalamus.  The hypothalamus, a
small region of brain, and the nearby pituitary gland produce a number of
important hormones, including many involved in reproduction and milk
production.

Other causes of galactorrhea-amenorrhea syndromes include hypothyroidism,
chronic use of dopamine antagonistic drugs (e.g., thorazine), and the
discontinuation of oral contraceptive regimens.

Related Disorders

Galactorrhea-amenorrhea syndromes include Chiari-Frommel Syndrome which is
associated with pregnancy and Ahumada-del Castillo Syndrome which is not
associated with pregnancy or large tumors.  (For more information on these
disorders, please choose "Chiari-Frommel" and "Ahumada" as your search terms
in the Rare Disease Database.)

Therapies:  Standard

Surgical removal of tumors usually resolves all symptoms of Forbes-Albright
Syndrome.  Smaller or inoperable tumors often respond to irradiation or
pharmacological treatment.  Drugs such as bromocriptine or lergotrile
mesilate lower prolactin levels, stopping the abnormal milk secretion, and
often restore menstrual functions.

Therapies:  Investigational

This disease entry is based upon medical information available through June
1989.  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 Forbes-Albright 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) 496-5751

References

THE MERCK MANUAL 15th ed:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 1029.