Werner Syndrome: A Rare Condition That Causes Premature Aging

Werner Syndrome is a disorder that affects children as early as adolescence. It may not be in full force until near age 30. It is a rare progressive disorder that makes the individual appear to age much faster than normal.

Key signs of Werner Syndrome include a high pitched voice, eye abnormalities (especially premature cataracts), abnormal insulin production, or impaired functioning of the testes or ovaries. Those with Werner Syndrome may also develop arteriosclerosis (loss of elasticity and thickening of the arterial walls). Some people may also become susceptible to developing tumors.

Those affected by Werner Syndrome grow abnormally slow and usually stop growing at puberty. Therefore, they will be particularly short and their weight will be low, even for their height. Graying hair and even hair loss are common by the age of twenty five.

As the premature aging caused by Werner Syndrome progresses, the individual will begin loosing the layer of fat just under the skin. Muscle tissue in certain areas may atrophy (waste away). The skin may also degenerate in certain areas, like the face, lower legs, feet, upper arms or hands. A pinched or beaked nose, and/or prominent eyes, is common as a result of this skin degeneration.

Those that have Werner Syndrome may be at increased risk for type 2 diabetes, osteoporosis, heart/artery disease, premature menopause, sarcoma, lymphoma and thyroid cancer.

Werner Syndrome is rare in general, but among premature aging disorders it is common. This condition is inherited if both parents pass on a particular defect in chromosome 8. Males and females are both affected. It is estimated that only one in one million people will develop Werner Syndrome.

Diagnosis of Werner Syndrome is difficult as there is no specific test for it. However, if the patient appears much older than they are, that is an important clue to doctors. Since diabetes can occur with this syndrome, checking blood sugar levels can aid in diagnosis. Checking cholesterol levels, which can reveal artery disease, helps put doctors on the right path as well.

Unfortunately specific treatments or cures for Werner Syndrome do not exist. Studies are being done with mice that have Werner Syndrome, which may shed some light on the subject.

Conditions that appear concurrently with the syndrome can be treated, however. If an individual with Werner Syndrome has diabetes, for example, the diabetes is treated in the same manner as it would be for other individuals. Any concurrent cancer can be treated with usual cancer treatments, except that radiation therapy is not used. Radiation therapy could be more harmful than helpful to those with Werner Syndrome.

Sources:
http://children.webmd.com/werner-syndrome
http://rarediseases.about.com/od/rarediseasesw/a/100304.htm
http://instruct1.cit.cornell.edu/Courses