Important
It is possible that the main title of the report Werner Syndromeis not the name you expected.

Synonyms

• Progeria of Adulthood
• WNS
• WS
• Atypical Werner Syndrome

Disorder Subdivisions

• None

General Discussion

Werner Syndrome is a rare progressive disorder that is characterized by the appearance of unusually accelerated aging (progeria). Although the disorder is typically recognized by the third or fourth decades of life, certain characteristic findings are present beginning during adolescence and early adulthood.

Individuals with Werner Syndrome have an abnormally slow growth rate, and there is cessation of growth at puberty. As a result, affected individuals have short stature and low weight relative to height. By age 25, those with the disorder typically experience early graying (canities) and premature loss of scalp hair (alopecia). As the disease progresses, additional abnormalities include loss of the layer of fat beneath the skin (subcutaneous adipose tissue); severe wasting (atrophy) of muscle tissue in certain areas of the body; and degenerative skin changes, particularly in the facial area, the upper arms and hands, and the lower legs and feet (distal extremities). Due to degenerative changes affecting the facial area, individuals with Werner Syndrome may have unusually prominent eyes, a beaked or pinched nose, and/or other characteristic facial abnormalities.

Werner Syndrome may also be characterized by development of a distinctive high-pitched voice; eye abnormalities, including premature clouding of the lenses of the eyes (bilateral senile cataracts); and certain endocrine defects, such as impaired functioning of the ovaries in females or testes in males (hypogonadism) or abnormal production of the hormone insulin by the pancreas and resistance to the effects of insulin (non-insulin-dependent diabetes mellitus). In addition, individuals with Werner syndrome may develop progressive thickening and loss of elasticity of artery walls (arteriosclerosis). Affected blood vessels typically include the arteries that transport oxygen-rich (oxygenated) blood to heart muscle (coronary arteries). Some affected individuals may also be susceptible to developing certain benign (noncancerous) or malignant tumors. Progressive arteriosclerosis, malignancies, and/or associated abnormalities may result in potentially life-threatening complications by approximately the fourth or fifth decade of life. Werner syndrome is inherited as an autosomal recessive trait.

Resources

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains
NY
10605
Tel: (914)428-7100
Fax: (914)997-4763
800: (888)663-4637
Askus@marchofdimes.com
http://www.marchofdimes.com

Progeria Research Foundation, Inc.
532 Lowell Street
Peabody
MA
01961-3453
USA
Tel: (978)535-2594
Fax: (978)535-5849
info@progeriaresearch.org
http://www.progeriaresearch.org

NIH/National Institute on Aging
PO Box 8057
Gaithersburg
MD
20892-8057
Tel: (301)496-1752
800: (800)222-2225
http://www.nih.gov/nia

NIH/National Institute of Allergy and Infectious Diseases
6610 Rockledge Drive
MSC 6612
Bethesda
MD
20892-6612
Tel: (301)496-5717
Fax: (301)402-3573
TDD: (800)877-8339
http://www.niaid.nih.gov/

International Registry of Werner Syndrome
University of Washington
Department of Pathology
Box 357470
Health Science Building K543
Seattle
WA
98195
Tel: (206)543-5088
Fax: (206)685-8356
nbhanson@u.washington.edu
http://www.wernersyndrome.org

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg
MD
20898-8126
Tel: (301)519-3194
Fax: (240)632-9164
800: (888)205-2311
TDD: (888)205-3223
gardinfo@nih.gov
http://www.genome.gov/10000409

Cancer.Net
American Society of Clinical Oncology
2318 Mill Road
Suite 800
Alexandria
VA
22314
Tel: (571)483-1780
Fax: (571)366-9537
800: (888)651-3038
contactus@cancer.net
http://www.cancer.net/patient