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Researchers first tended to believe
that it was part of normal ageing. They have now dismissed the
theory since not all the sense organs are affected, nor is there
any sign of senility or ageing of the central nervous system.
Two types
There are two major types of progeria. The first one is known as
rare Hutchinson-Gilford syndrome and it is a very rare occurrence.
It sets in in early childhood. The second type is called Werner's
syndrome. This condition sets in later in life. There is a third
variety too, where progeria occurs with Hallerman-Streiff-François
syndrome. In this type, dwarfism is a prominent feature.
The Hutchinson-Gilford syndrome
Infant progeria or Hutchinson-Gilford syndrome usually sets in
when the child is about one-year-old. The child may have had a
perfectly normal infancy until the onset of infant progeria.
What happens
Children affected with infant progeria seldom grow beyond the
size of a normal five-year-old and their physical appearance is
similar to that of a sixty-year-old by the time they complete ten
years of age. Signs of baldness or thinning hair, thinning of the
skin, prominence of blood vessels of the scalp and vascular diseases
occur. While most children have normal intelligence, some of them
may be mentally retarded. Some children may even be precocious.
Before the child is ten, he may develop heart diseases and extensive
arteriosclerosis.
Cause
The cause for infant progeria has not been established. Although
some researchers suspected hereditary factors, they have been unable
to draw conclusive proofs.
Werner’s syndrome
Werner’s syndrome, which is also called progeria of the adult,
usually occurs in young adulthood. In rare cases, it may set in in
late adolescence. Here the ageing changes are less pronounced than
in the case of infant progeria and a person affected appears thirty
older than his natural age. Since body growth is already complete,
there is no dwarfism; a person may be shorter than average.
Here too, the patient has premature graying
or balding of the hair. Besides, he suffers loss of hearing and
teeth. He also has an acute tendency to develop cataract, acute
arthritic episodes, skin ulcers and osteoporosis. Chances of
developing heart disease, diabetes mellitus and cancer are very
high.
Although the exact cause of Werner’s
syndrome has not been established, it has been proved that it is
hereditary and is transmitted as a recessive trait. |
