Merry Syndrome
| Classification according to ICD-10 | |
|---|---|
| E23.6 | Other pituitary disorders Adiposogenital dystrophy |
| ICD-10 online (WHO version 2019) | |
The Fröhlich syndrome (also hypothalamic syndrome , Babinski-Fröhlich syndrome or dystrophia adiposogenitalis as well as Fröhlich's disease ) is a syndrome that is associated with obesity ( adiposity ) female fat distribution type, short stature and other endocrine disorders. This rare disease is caused by a pituitary or hypothalamus tumor and the associated hypothalamic disorder.
Symptoms
The main symptom is obesity with a preferred increase in fat deposits on the hips, thighs and stomach (female fat distribution pattern) paired with an underdevelopment of the gonads ( gonads ). The latter can manifest itself in delayed or even absent puberty ( pubertas tarda ).
If the disease only occurs in adulthood, it can also lead to regression of the genital organs and infertility. This can be accompanied by short stature , headaches , mental development retardation, visual disturbances and increased thirst ( polydipsia ) due to increased urination ( polyuria ) in the sense of central diabetes insipidus .
These symptoms are due to the mass of the tumor in the hypothalamus. This part of the brain is responsible for the regulation of the pituitary gland, the central control organ of the peripheral hormonal glands in humans (adrenal gland, testes or ovary, thyroid gland); it is also important for the integration and regulation of eating and drinking behavior, sexual behavior, and autonomic functions of the internal organs ( homeostasis ).
A specific hormone of the hypothalamus is the antidiuretic hormone (ADH), the failure of which explains the symptoms of increased urination in Fröhlich's syndrome.
Laurence-Moon-Biedl-Bardet syndrome shows a similar clinical picture .
history
The syndrome, named after Alfred Fröhlich , was first described by Joseph Babinski in 1900 . The original works were JF Babinski: Tumeur du corps pituitaire sans acromégalie et avec arrêt de développement des organes génitaux . Revue neurologique 1900 and A. Fröhlich: A case of tumor of the hypophysis cerebri without acromegaly. Wiener Klinische Rundschau 1901. The term has largely disappeared from medical literature since the 1950s.
Older literature
- Ludwig Weissbecker: Diseases of the pituitary-diencephalon system. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 1008-1013, here: p. 1011 f .: Dystrophia adiposo-genitalis (Fröhlich's disease) and the Lawrence-Moon-Biedl syndrome .
