Adiposogenital dystrophy of Babinsky-Frohlich

With adipozogenital dystrophy Babinsky-Frohlich observed predominant deposition of fat on the trunk, especially in the abdomen ("apron") and hips. As a rule, it develops in the period preceding puberty.

[1], [2]

The causes of adiposogenital dystrophy Babinsky-Frohlich

The most common causes of typical adiposogenital dystrophy are craniopharyngioma and the chromophobic adenoma of the pituitary gland, as well as other tumor processes that extend to the ventromedial hypothalamus and medial elevation. It can develop as a result of the defeat of the hypothalamic-pituitary system by the tuberculosis process (now extremely rare), with encephalitis, with severe hydrocephalus. Sometimes an obvious cause of the disease can not be identified. In this case, the disease is usually of a family nature. The manifestation of constitutional predisposition is promoted by inappropriate diet and sedentary lifestyle, chronic emotional stress, severe somatic and infectious diseases.

Pathogenesis of adiposogenital dystrophy of Babinsky-Frohlich

Pathological changes are associated with hormonal dysfunction and a violation of biological motivations. The pathology of the release of the releasing factors of the hypothalamus is revealed, which leads to a violation of the secretion of ACTH, STH, TTG, gonadotropins. Motivation disorders are determined by the dysfunction of cerebral systems, which determine food motivation, in which the leading role is assigned to the ventromedial and ventrolateral nuclei of the hypothalamus. Depending on the etiology, a primary lesion of the pituitary gland or hypothalamus is possible.

Symptoms of Adiposogenital Dystrophy Babinsky-Frohlich

Characteristic growth retardation, hypogonadism. In men, underdevelopment of the genitals is often accompanied by cryptorchidism. Sexual organs in women are diminished, hypoplastic; Has a method of amenorrhoea. There is no hair covering of the skin, hair on the head is rare, with a tendency to baldness. There is a lag in the ossification of the epiphyses, flat feet and X-shaped legs. Characterized by mental underdevelopment, emotional flattening. Sexual desire is reduced or absent. If the disease begins after puberty, then there are no ossifications and the sex signs are sufficiently developed. As the disease develops due to atrophy of the sexual glands, amenorrhea, a decrease in sexual desire, impotence occur.

Often, the dystrophy of Babinsky - Frohlich combined with hypothyroidism of the thyroid gland, violations of water-salt metabolism, hyperthermia, hypersomnia, signs of acromegaly. Often observed trophic changes in the skin (keratosis, pigmentation, keratitis). There may be erased forms that flow like a mixed form of cerebral obesity with a distribution of fat close to the form of adiposogenital dystrophy.

Differential diagnosis

It is necessary to differentiate from obesity with eunuchoidism. Facilitate diagnosis is the presence of signs of CNS damage and the absence of eunuchoid body proportions.

[3], [4], [5]

Treatment of adiposogenital dystrophy Babinsky-Frohlich

Treatment of adipozogenital dystrophy Babinsky-Frohlich depends on the causes that caused the disease. When the constitutional inferiority is decompensated, the tactic of therapeutic intervention is the same as in the mixed form of cerebral obesity. When expressed hypogonadism, alternate courses of treatment with gonadotropic drugs in boys in combination with the use of testosterone are used. Treatment is long - an average of 1-2 years. Hormone replacement therapy should be administered in conjunction with endocrinologists.