yogabook / pathologie / fröhlich syndrome

fröhlich syndrome / hypothalamic syndrome / Babinski-Fröhlich syndrome / dystrophia adiposogenitalis

Definition of

A very rare syndrome caused by a disorder of the pituitary gland with severe obesity of the female fat distribution type, pituitary dwarfism and other endocrine disorders. A striking feature is the increase in fat deposits on the thighs, hips and abdomen (female fat distribution pattern) combined with underdevelopment of the gonads (gonads), which causes puberty to be absent or delayed ((pubertas tarda). There is a reduced production of the important control hormones: TRH (thyrotropin-releasing hormone, leads to hypothyroidism), CRH (corticotropin-releasing hormone, leads to decreased release of cortisol, sex hormones, aldosterone), GNrH (gonadotropin-releasing hormone, leads to decreased LH and FSH and thus to gonadal dysfunction), GHRH (growth hormone releasing hormone) somatostatin (growth hormone inhibiting hormone), prolactin (reduced milk production) vasopressin (water balance disorders), leptin (reduced production leads to a suppressed feeling of satiety). If the disease first occurs in adults, it often leads to infertility. If it starts at a young age, it results in pituitary dwarfism. The loss of the hormone ADH leads to diabetes insippidus (polyuria, polydipsia). Mental developmental delays (mental retardation, reduced intelligence) and visual disturbances are also observed. Affected thyroid function causes massive obesity. The syndrome predominantly affects males.

Cause

1. Hypothalamic tumor whose mass also affects the pituitary gland

Diagnosis

1. Clinical
2. CT to confirm the tumor

Symptoms

1. Pronounced symptoms of diabetes insippidus: unquenchable thirst (pronounced polydipsia, corresponding polyuria)
2. constant hunger
3. onset before puberty: delayed onset or absence of puberty
4. Visual disturbances
5. Symptoms of hypothyroidism
6. Pronounced obesity due to the combination of suppressed satiety and hypothyroidism
7. Delayed or absent puberty, impaired sexual function
8. Lower life expectancy

Therapy

1. OP, substitution, symptomatic