Diseases & Conditions


Werner Syndrome

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

Disorder Subdivisions

    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.

    Organizations related to Werner Syndrome
    • Cancer.Net
      American Society of Clinical Oncology
      Alexandria VA 22314
      Phone #: 571-483-1780
      800 #: 888-651-3038
      e-mail: [email protected]
      Home page: http://www.cancer.net/patient
    • Genetic and Rare Diseases (GARD) Information Center
      PO Box 8126
      Gaithersburg MD 20898-8126
      Phone #: 301-519-3194
      800 #: 888-205-2311
      e-mail: [email protected]
      Home page: http://www.genome.gov/10000409
    • International Registry of Werner Syndrome
      University of Washington
      Seattle WA 98195
      Phone #: 206-543-5088
      800 #: --
      e-mail: [email protected]
      Home page: http://www.wernersyndrome.org
    • March of Dimes Birth Defects Foundation
      1275 Mamaroneck Avenue
      White Plains NY 10605
      Phone #: 914-428-7100
      800 #: 888-663-4637
      e-mail: [email protected]
      Home page: http://www.marchofdimes.com
    • NIH/National Institute of Allergy and Infectious Diseases
      6610 Rockledge Drive
      Bethesda MD 20892-6612
      Phone #: 301-496-5717
      800 #: --
      e-mail: N/A
      Home page: http://www.niaid.nih.gov/
    • NIH/National Institute on Aging
      PO Box 8057
      Gaithersburg MD 20892-8057
      Phone #: 301-496-1752
      800 #: 800-222-2225
      e-mail: N/A
      Home page: http://www.nih.gov/nia
    • Progeria Research Foundation, Inc.
      532 Lowell Street
      Peabody MA 01961-3453
      Phone #: 978-535-2594
      800 #: --
      e-mail: [email protected]
      Home page: http://www.progeriaresearch.org

    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html