CD-ROM Today 1996 January/CD-ROM Today 1996 January.iso/dp/0384/03843.txt

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$Unique_ID{BRK03843}
$Pretitle{}
$Title{Hyperostosis Frontalis Interna}
$Subject{Hyperostosis Frontalis Interna Endostosis Crani Hyperostosis
Calvariae Interna Morgagni-Stewart-Morel Syndrome Acromegaly Ieontiasis Ossea
Paget's Disease Leontiasis Ossea Virchow's Disease Crouzon Disease
Galactorrhea Myotonic Dystrophy Diabetes Insipidus}
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Copyright (C) 1992 National Organization for Rare Disorders, Inc.

869:
Hyperostosis Frontalis Interna

** IMPORTANT **
It is possible that the main title of the article (Hyperostosis Frontalis
Interna) is not the name you expected.  Please check the SYNONYMS listing to
find the alternate name and disorder subdivisions covered by this article.

Synonyms

     Endostosis Crani
     Hyperostosis Calvariae Interna
     Morgagni-Stewart-Morel Syndrome

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

     Acromegaly
     Ieontiasis Ossea
     Paget's Disease
     Leontiasis Ossea (also known as Virchow's Disease)
     Crouzon Disease
     Galactorrhea
     Myotonic Dystrophy
     Diabetes Insipidus

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.

Hyperostosis Frontalis Interna is a disorder in which there is excessive
growth or thickening of the frontal bone of the skull.  It is not known if
this disorder is actually rare.  Some scientists feel that this may be a
common abnormality found in as many as 12% of the female population.  This
disorder has been found in association with a variety of conditions such as
seizures, headaches, obesity, diabetes insipidus, excessive hair growth and
sex gland disturbances.  Increased serum alkaline phosphatase and elevated
serum calcium may occur.

Symptoms

The major feature of Hyperostosis Frontalis Interna is excessive growth or
thickening of the frontal bone of the head.  This excess growth can only be
seen in an x-ray.  As a result, scientists feel that this condition may be
much more prevalent than suspected, but often goes undetected.  Many people
have no apparent symptoms.

Other conditions that may be found in patients with this disorder are:
obesity, a condition in which secondary male sexual traits are acquired by a
female (virilization); a central nervous system disorder characterized by a
sudden, aimless, uncontrollable discharge of electrical energy in the brain
causing a convulsion or loss of consciousness (epilepsy); decreased vision;
headaches; disturbances of the ovaries and testes (sex glands or gonads);
excessive body hair; and/or diabetes.  (For more information on these
disorders, choose "Epilepsy" and/or "Diabetes" as your search terms in the
Rare Disease Database).

Causes

Hyperostosis Frontalis Interna has been found in multiple generations
suggesting that the disorder may be inherited as a dominant trait.  It is not
known if the disorder is autosomal dominant or X-linked.  There are no known
cases of male-to-male (father to son) transmission.

Human traits, including the classic genetic diseases, are the product of
the interaction of two genes, one received from the father and one from the
mother.

In dominant disorders a single copy of the disease gene (received from
either the mother or father) will be expressed "dominating" the other normal
gene and resulting in the appearance of the disease.  The risk of
transmitting the disorder from affected parent to offspring is fifty percent
for each pregnancy regardless of the sex of the resulting child.

Affected Population

Hyperostosis Frontalis Interna affects females 9 times more often than males.
This disorder presents itself most often among the middle-aged and elderly
but has also been found in adolescents.

Related Disorders

Symptoms of the following disorders can be similar to those of Hyperostosis
Frontalis Interna.  Comparisons may be useful for a differential diagnosis:

Acromegaly is a slowly progressive, chronic metabolic disorder in which
an excess of growth hormone causes abnormal enlargement of various tissues of
the body and unusual height.  Most conspicuously affected are the
extremities, jaws, and face.  The enlargement of soft tissue, especially of
the heart, is a serious feature of this disorder.  High blood pressure
(hypertension) may be another serious consequence of Acromegaly.  (For more
information on this disorder choose "Acromegaly" as your search term in the
Rare Disease Database).

Paget's Disease is a slowly progressive disease of the skeletal system
characterized by abnormally rapid bone breakdown and formation, leading to
the development of bones that are dense but fragile.  This disorder usually
affects middle-aged and elderly people and most frequently occurs in the
spine, skull, pelvis, thighs and lower legs.  When it occurs in the skull it
can cause hearing loss.  (For more information on this disorder choose
"Paget's" as your search term in the Rare Disease Database).

Leontiasis Ossea or Virchow's Disease is a disorder in which there is an
overgrowth of the bones of the face and sometimes of the cranium.  This
disorder causes a general enlargement and distortion of all the features.

The following disorders have been found in association with Hyperostosis
Frontalis Interna.  They are not necessary for a differential diagnosis:

Crouzon Disease is a genetic disorder characterized by abnormalities in
the skull, face, and brain caused by premature hardening of the skull.  The
skull is made up of several bony plates initially joined by fibrous
connective tissue which normally fuse together and harden over a period of
several years after growth of the brain.  Facial deformities are often
present at birth and may progress with time.  Vision disturbances and
deafness may develop in some cases.  (For more information on this disorder
choose "Crouzon" as your search term in the Rare Disease Database).

Galactorrhea is a condition in which there is a spontaneous flow of milk
from the nipple.

Myotonic Dystrophy is an inherited disorder involving the muscles,
vision, and endocrine glands.  It can cause mental deficiency and loss of
hair.  Onset of this disorder commonly occurs during young adulthood although
it can occur at any age and is extremely variable in degree of severity.
Symptoms of this disorder may be tripping, falling, difficulty in moving the
neck, lack of facial expression and a nasal sounding voice.  (For more
information on this disorder choose "Myotonic Dystrophy" as your search term
in the Rare Disease Database).

Diabetes Insipidus is due to an abnormality of anti-diuretic hormone
(vasopressin or ADH) originating in the posterior lobe of the pituitary gland.
The lack of this hormone on the kidney causes excretion of excessive
quantities of very dilute (but otherwise normal) urine.  Excessive thirst is
the major symptom of this disorder.  (For more information on this disorder,
choose "Diabetes Insipidus" as your search term in the Rare Disease
Database.)

Therapies:  Standard

There is no known treatment for Hyperostosis Frontalis Interna.  Seizures and
headaches can be treated with standard medications.

Genetic counseling may be of benefit for patients and their families.
Other treatment is symptomatic and supportive.

Therapies:  Investigational

This disease entry is based upon medical information available through April
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 Hyperostosis Frontalis Interna, please contact:

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

     National Craniofacial Foundation
     3100 Carlisle Street, Suite 215
     Dallas, TX  75204
     [phone]

     National Association for the Craniofacially Handicapped
     P.O. Box 11082
     Chattanooga, TN  37401
     [phone]

     The National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
     Box AMS
     Bethesda, MD  20892
     [phone]

For Genetic Information and Genetic Counseling Referrals:

     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

MENDELIAN INHERITANCE IN MAN, 9th Ed.:  Victor A. McKusick, Editor:  Johns
Hopkins University Press, 1990.  Pp. 493.

BIRTH DEFECTS ENCYCLOPEDIA, Mary Louise Buyse, M.D., Editor-In-Chief;
Blackwell Scientific Publications, 1990.  Pp.  909-910.