Babinski-Fröhlich syndrome
Related people
Condition characterized by feminine obesity and sexual infantilism, atrophy or hypoplasia of the gonads, and altered secondary sex characteristics. Headaches, retarded growth and sexual development; mental retardation, visual troubles; polyuria; polydipsia. It is usually associated with tumours of the hypothalamus, causing increased appetite and depressed secretion of gonadotropin.
Although the syndrome may appear in childhood, it occurs most often during puberty, occasionally in the postadolescent period.
Many overweight children may appear to have the disorder because of the concurrence of obesity and retarded sexual development; these children have no endocrine disturbances, however, and they mature normally after delayed puberty.
The adult equivalent is called pituitary infantilism. Either sex may be affected. Prevalent in males. No hereditary or familial tendencies are present.
The disease is also named for Alfred Fröhlich, the Austrian neurologist who first described its typical pattern.
First described by Morgagni.
Bibliography
- J. F. Babinski:
Tumeur du corps pituitaire sans acromégalie et avec arrêt de développement des organes génitaux.
Revue neurologique, Paris, 1900, 8: 531-535. - A. Fröhlich:
Ein Fall von Tumor der Hypophysis cerebri ohne Akromegalie.
Wiener klinische Rundschau, 1901, 15: 833-836; 906-908. - P. E. Launois, M. Cléret:
Le syndrome hypophysaire adiposogénital.
Gazette des hôpitaux, Paris, 1910, 83: 57-64, 83-86.