Diseases of Accelerated & Premature Aging

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Certain abnormal diseases and conditions can cause premature and accelerated aging. The conditions that normally occur when one is in his seventies may occur even in one’s teens.

Progeroid Syndromes: The progeroid syndromes are conditions that produce premature aging and a shortened life expectancy. The most striking feature of these rare disorders is extremely accelerated aging. Affected children develop all of the external signs of old age, including baldness, hunched posture, and dry, wrinkled skin. Unlike normal aging, however, progeroid syndromes also include such features as lack of ovarian or testicular activity (including sterility and absence of menstrual periods) and unusually short stature. Thus, progeria is not an exact model of accelerated aging.

Two forms of progeroid syndromes are Hutchinson-Gilibrd syndrome (commonly referred to as progeria), which begins in early childhood, and Wemer’s syndrome, which begins in adolescence or early adult life. Both syndromes are hereditary, but the exact genetic basis of Hutchinson-Gilford syndrome remains undetermined. However, recent advances have been made in understanding the genetic basis of Wemer’s syndrome. Hutchinson-Gilford syndrome produces baldness, and other conditions normally associated with aging (for example, diseases of the heart, kidneys, and lungs).  Similarly, Werner’s syndrome produces the skin changes of scleroderma and baldness and a high rate of atherosclerosis. The central nervous system, and therefore daily activities dependant on brain function, is largely spared in both Hutchinson-Gilford syndrome and Wemer’s syndrome, unless an affected person hits a stroke. Other features of Wemer’s syndrome include premature cataracts, muscle wasting, and a high rate of cancer (including some types that arc rare in unaffected people). Down Syndrome is more common than the progeroid syndromes. Down syndrome also produces in younger adults conditions typical of old age, including glucose intolerance, blood vessel disease, a high rate of cancer, hair loss, degenerative bone disease, and premature death.

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