Babinski-Frelich adiposogenital dystrophy

In Babinski-Fröhlich adiposogenital dystrophy, there is a predominant deposition of fat on the trunk, especially in the abdominal area ("apron") and thighs. As a rule, it develops in the period preceding puberty.

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Causes of Babinski-Froelich adiposogenital dystrophy

The most common causes of typical adiposogenital dystrophy are craniopharyngioma and chromophobe adenoma of the pituitary gland, as well as other tumor processes spreading to the ventromedial hypothalamus and median eminence. It can develop as a result of damage to the hypothalamic-pituitary system by a tuberculous process (currently extremely rare), with encephalitis, with severe hydrocephalus. Sometimes an obvious cause of the disease cannot be identified. In such cases, the disease usually has a familial nature. The manifestation of constitutional predisposition is facilitated by an inappropriate diet and a sedentary lifestyle, chronic emotional stress, severe somatic and infectious diseases.

Pathogenesis of adiposogenital dystrophy Babinski-Froelich

Pathological changes are associated with hormonal dysfunction and disruption of biological motivations. Pathology of the secretion of hypothalamic releasing factors is revealed, leading to disruption of the secretion of ACTH, STH, TSH, gonadotropins. Motivational disorders are determined by dysfunction of the cerebral systems that determine food motivation, in which the leading role is given to the ventromedial and ventrolateral nuclei of the hypothalamus. Depending on the etiology, predominant damage to the pituitary gland or hypothalamus is possible.

Symptoms of Babinski-Froelich adiposogenital dystrophy

Characteristic features include growth retardation and hypogonadism. In men, underdevelopment of the genitals is often accompanied by cryptorchidism. In women, the genitals are reduced in size and hypoplastic; there is a meta-menorrhea. There is no hair growth on the skin, the hair on the head is sparse, with a tendency to baldness. There is a delay in the ossification of the epiphyses, flat feet and knock-knees. Characteristic features include mental retardation and emotional flattening. Sexual desire is reduced or absent. If the disease begins after puberty, there are no ossification disorders and sexual characteristics are sufficiently developed. As the disease progresses, amenorrhea, decreased sexual desire and impotence occur due to atrophy of the sex glands.

Babinski-Frohlich dystrophy is often combined with hypothyroidism, water-salt metabolism disorders, hyperthermia, hypersomnia, and signs of acromegaly. Trophic changes in the skin (keratosis, pigmentation, keratitis) are often observed. There may be latent forms that occur as a mixed form of cerebral obesity with fat distribution close to the form of adiposogenital dystrophy.

Differential diagnosis

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

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Treatment of adiposogenital dystrophy Babinski-Froelich

Treatment of adiposogenital dystrophy of Babinski-Fröhlich depends on the causes of the disease. In case of decompensation of constitutional inferiority, the tactics of therapeutic intervention are the same as in case of mixed form of cerebral obesity. In case of pronounced hypogonadism, alternating courses of treatment with gonadotropic drugs in boys in combination with testosterone are used. The treatment is long-term - on average 1-2 years. Hormonal replacement therapy should be prescribed together with endocrinologists.