Adiposogenital dystrophy

Adiposogenital dystrophy
Classification and external resources
Specialty	endocrinology
ICD-10	E23.6
ICD-9-CM	253.8
DiseasesDB	29318

Adiposogenital dystrophy is a condition which may be caused by tertiary hypogonadism originating from decreased levels in GnRH. Low levels of GnRH has been associated with defects of the feeding centers of the hypothalamus, leading to an increased consumption of food and thus caloric intake.

Presentation

It is characterized by:

• Obesity
• Growth retardation and retarded sexual development, atrophy or hypoplasia of the gonads, and altered secondary sex characteristics,
• Headaches
• Problems with vision
• polyuria, polydipsia.

It is usually associated with tumors of the hypothalamus, causing increased appetite and depressed secretion of gonadotropin. It seems to affect males mostly.

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.

Synonyms

It has several other names:^[1]

• Babinski-Fröhlich syndrome^[2] (named after Joseph Babinski^[3] and Alfred Fröhlich,^[4] but probably first described by Morgagni). (It was given its name by Harvey Cushing.)^[5] commonly associated with slipped capital femoral epiphysis
• Froelich's syndrome
• Frölich's Syndrome
• Hypothalamic Infantilism-Obesity
• Launois-Cleret Syndrome
• Sexual Infantilism

See also

• Prader-Willi syndrome, which it might resemble. However, this is a genetic disorder.

References

1. National Organisation for Rare Disorders - Froelich's syndrome
2. synd/1792 at Who Named It? - Babinski-Fröchlich syndrome
3. J. F. Babinski. Tumeur du corps pituitaire sans acromégalie et avec arrêt de développement des organes génitaux. Revue neurologique, Paris, 1900, 8: 531-535.
4. A. Fröhlich. Ein Fall von Tumor der Hypophysis cerebri ohne Akromegalie. Wiener klinische Rundschau, 1901, 15: 833-836; 906-908.
5. Zárate A, Saucedo R (2007). "[The adiposogenital distrophy or Frohlich syndrome and the beginning of the concept of neuroendocrinology]". Gac Med Mex (in Spanish) 143 (4): 349–50. PMID 17969845.