$Unique_ID{BRK03590}
$Pretitle{}
$Title{Chiari-Frommel Syndrome}
$Subject{Chiari-Frommel Syndrome Lactation with Atrophy of Uterus
Lactation-Uterus Atrophy Chiari's I Syndrome Frommel-Chiari Syndrome}
$Volume{}
$Log{}

Copyright (C) 1986, 1989 National Organization for Rare Disorders, Inc.

146:
Chiari-Frommel Syndrome

** IMPORTANT **
It is possible that the main title of the article (Chiari-Frommel
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

     Lactation with Atrophy of Uterus
     Lactation-Uterus Atrophy
     Chiari's I Syndrome
     Frommel-Chiari 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.


Chiari-Frommel Syndrome is an endocrine disorder in which lactation
(discharge of milk) and absence of ovulation and menstruation persists for an
unusually long time after childbirth.  It is thought to result from an
abnormality in the pituitary gland or the hypothalamic region of the brain.
Spontaneous recovery sometimes occurs.

Symptoms

After a normal pregnancy and childbirth, with an uneventful period of
lactation, the woman affected with Chiari-Frommel Syndrome finds that her
menses fail to resume and that there is a persistent discharge from her
nipples.  This may be light or heavy, and continue for months to years.
Headache, backache, abdominal pain, depression or extreme moodiness, and
constriction of the visual fields may occur.  Some affected women may become
obese.

The uterus shrinks in size due to the absence of normal hormonal cycles.
Laboratory tests reveal persistently high levels of prolactin (the hormone
which stimulates milk production after childbirth), high levels of Luteinizing
Hormone, and low levels of Follicle Stimulating Hormone (involved in the
ovulatory cycle).

Causes

The exact causes of Chiari-Frommel Syndrome are not known, although the
disease clearly represents some abnormality of the hypothalamic-pituitary
axis.  This system consists of the endocrine gland known as the anterior
pituitary, which produces the hormones, mentioned above, and a small region
of the brain called the hypothalamus, which regulates the secretions of the
pituitary gland.

Most cases of Chiari-Frommel Syndrome appear to result from pituitary
tumors.  Tiny lesions in the hypothalamus may be involved occasionally.  Oral
contraceptives may also be associated with this disorder.

Related Disorders

Other syndromes in which there is excessive milk production (galactorrhea)
and absence of menstruation (amenorrhea) include Ahumada-del Castillo
Syndrome and Forbes-Albright Syndrome.  The former of these occurs without
pregnancy, for unknown reasons; the latter results specifically from a tumor
in the pituitary gland.  (For more information on these disorders, choose
"Ahumada" and "Forbes-Albright" as your search terms in the Rare Disease
Database.

Therapies:  Standard

Chiari-Frommel Syndrome may be difficult to treat if very small pituitary
tumors are responsible for the disorder.  Larger tumors may often be removed
surgically.  In many cases, prolactin levels can be reduced, and ovulatory
cycles restored, with bromocriptine.

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 Chiari-Frommel 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.  P. 1029.