$Unique_ID{BRK03756}
$Pretitle{}
$Title{Froelich's Syndrome}
$Subject{Froelich's Syndrome Adiposogenital Dystrophy Dystrophia
Adiposogenitalis Sexual Infantilism Babinski-Froehlich's Syndrome Hypothalamic
Infantilism - Obesity Leaunois-Cleret Syndrome}
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Copyright (C) 1986, 1989 National Organization for Rare Disorders, Inc.

102:
Froelich's Syndrome


** IMPORTANT **
It is possible that the main title of the article (Froelich's 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

     Adiposogenital Dystrophy
     Dystrophia Adiposogenitalis
     Sexual Infantilism
     Babinski-Froehlich's Syndrome
     Hypothalamic Infantilism - Obesity
     Leaunois-Cleret 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.


Froelich's syndrome, or adiposogenital dystrophy, is characterized by
delayed puberty, small testes in males, and obesity.  It affects primarily
males.  In recent years, Froelich's syndrome has come to refer exclusively to
such features in boys who have lesions in the hypothalamic region of the
brain.  (Different parts of the hypothalamus control sexual development and
appetite.)  Teenagers with this disorder must be differentiated from those who
simply have a familial trait of slower than normal growth or Prader-Willi
Syndrome.

Symptoms

Puberty is considered delayed if the external genitalia fail to enlarge
normally by about the age of thirteen and a half, or if the period between
the beginning and the completion of the growth of the genitalia (in girls,
breast development and the first menstruation) takes longer than 5 years.

In Froelich's Syndrome, obesity accompanies such a delay.  Body growth
may also be slow, and patients often remain short in stature.  Secondary
sexual characteristics such as facial and body hair, voice changes,
coarsening of the skin, etc., appear very slowly.  Finger nails are often
malformed or undersized.  Headaches are common.  Some patients may have mental
retardation, visual disturbances, and, rarely, diabetes mellitus.

Causes

In some cases, the anterior pituitary, an endocrine gland attached to the
brain, fails to secrete the hormones necessary for puberty to proceed because
of some lesion to the gland.  Froelich's syndrome is due to lesions in the
hypothalamus, one part of which produces substances that stimulate the
pituitary; another area of the hypothalamus is believed to regulate the
appetite.  Tumors and inflammation due to infections such as tuberculosis or
encephalitis usually cause the lesions.

Related Disorders

Hypogonadotropic hypogonadism is a disorder of development of the region of
the hypothalamus that regulates the pituitary hormones (gonadotropins) that
in turn regulate the functioning of the gonads.  In the congenital Laurence-
Moon-Biedl syndrome, the child has defects in the ears and/or eyes, mental
retardation, anomalies of the fingers and toes, obesity, and underdevelopment
of the genitalia.  In Prader-Willi syndrome, also congenital, neurological
and behavioral abnormalities accompany hypogonadism and obesity.  (For more
information on these disorders, choose "Laurence-Moon" and "Prader-Willi" as
your search terms in the Rare Disease Database).

Therapies:  Standard

Pituitary extracts may be administered to replace the missing hormones in
patients with Froelich's Syndrome.  Hypothalamic tumors should be removed if
possible, although this will not restore destroyed tissue.  Appetite may be
very difficult to manage, although weight control depends on this.

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 Froelich's 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 Neurological Disorders & Stroke (NINDS)
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-5751
     (800) 352-9424

     Short Stature Foundation
     17200 Jamboree Rd., Suite J
     Irvine, CA  92714-5828
     (714) 474-4554

     The Magic Foundation
     1327 N. Harlem Ave.
     Oak Park, IL  60302
     (708) 383-0808

     Human Growth Foundation
     7777 Leesburg Pike
     P.O. Box 3090
     Falls Church, VA  22043
     (703) 883-1773
     (800) 451-6434

References

Regulation of the endocrine hypothalamus.  Reichlin, S. Med Clin North
Am 1978 Mar; 62(2):235-50.