Fröhlich's syndrome

Fröhlich's syndrome

Etymology

The disease is named for Alfred Fröhlich, the Austrian neurologist who first described its typical characteristics.

Overview

More commonly referred to as Adiposogenital Dystrophy, Fröhlich's syndrome is a relatively rare childhood metabolic disorder that occurs most frequently in males. It is most often associated with tumours of the hypothalamus or the pituitary gland.

Characteristics

Fröhlich's syndrome is characterized by one or a combination of the following:

• obesity
• mental retardation
• growth retardation
• retarded development of the genital organs (genital hypoplasia)
• changes in secondary sex characteristics
• increased appetite
• depressed secretion of gonadotropin
• headaches
• visual impairment
• polyuria
• polydipsia

Human chorionic gonadotropin (HCG) is frequently used to treat the syndrome. Interestingly enough, during the diet-crazed 50s and 60s, the hope that administering HCG to non-syndromic individuals would lead to weight-loss was shattered following some unpleasant reactions to unwarranted administration of the hormone.

In some overweight children a false diagnosis of Fröhlich's syndrome may arise when delayed sexual development is also observed. A lack of associated endocrine disturbance is sufficient to eliminate this misdiagnosis and these children normally progress to normal maturation.

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