Isenko-Cushing syndrome

Distinguish Itenko-Cushing's disease, which has a hypothalamic-pituitary origin, and the actual Itenko-Cushing syndrome is a disease associated with the primary defeat of the adrenal glands. In this article, only the cerebral form of the Itenko-Cushing disease is considered.

[1], [2], [3], [4], [5]

Causes of Itenko-Cushing syndrome

In addition to ACTH-secreting pituitary tumors (usually basophilic adenomas), the disease rarely develops as a result of neuroinfectious or post-traumatic damage to the hypothalamic-pituitary region. Decompensation of institutional dysfunction of the hypothalamic-pituitary-adrenal axis is often detected, under the influence of chronic stress, hormonal changes, intoxications, and infectious diseases.

[6], [7], [8], [9], [10]

Pathogenesis of Itenko-Cushing syndrome

The clinical picture is determined by the increase in plasma levels of ACTH and cortisol. Hypertrophy of the cortical layer of the adrenal gland is secondary centrally determined. Hyperproduction ACTH can be defined as the predominant pathology of the hypothalamus (excessive production of corticotropin-releasing factor) and primary pituitary lesions (ACTH-secreting adenomas of the pituitary gland). It is believed that in the genesis of ACTH-secreting adenomas of the pituitary gland the decisive role is played by the previous prolonged stimulation by the excessively produced corticotropin releasing factor.

Symptoms of Itenko-Cushing syndrome

There is a selective localization of fat on the face (moonlike face), chest, abdomen, neck, above the VII cervical vertebra ("climacteric hump") in combination with relatively thin limbs. The face is purple-red, the skin is dry, scaly, numerous acne. Characterized by wide dystrophic stretch bands, usually purple-cyanotic color on the skin of the abdomen, hips, shoulders, mammary glands. In the clinical picture - arterial hypertension, diabetes mellitus, hirsutism combined with hair thinning on the head, osteoporosis and compression fractures, muscle weakness, fragility of capillaries, poor healing of wounds, reduced resistance to infections.

Psychopathological disorders are of a different nature - from hypersomnia to insomnia, from euphoria to depression, sometimes true psychoses take place. There is a change in morphological and functional sexual characteristics. In women, in addition to hirsutism, there is a coarsening of the voice, amenorrhea or oligomenorrhea, decreased sexual desire, masculinization. In men, feminization, gynecomastia, testicular hypotrophy, lowering of sexual desire and spermatogenesis are sometimes observed. Often, the skin of patients in areas undergoing pressure around the nipples and in the places of scar formation darkens.

Differential diagnosis of Isenko-Cushing syndrome

In the presence of signs of hyperadrenocorticism, it is necessary to exclude the primary pathology of the adrenal glands, ie, the Itenko-Cushing syndrome. For this purpose, the levels of ACTH and cortisol in the blood are determined and a dexamethasone test is performed. The test with dexamethasone is based on the suppression of endogenous ACTH production on the principle of feedback. With Isenko-Cushing's disease, dexamethasone is administered internally 2 mg every 6 hours for 2 days, excretion in the urine of 17-ACS decreases by more than 50%; Cushing's syndrome excretion of 17-ACS in the urine does not change.

It is necessary to exclude cases with ectopic production of ACTH in small-cell lung cancer, carcinoid tumors, tumors of pancreatic islets. With ectopic tumors, the course of the syndrome is more malignant, there is no significant increase in body weight, hypokalemia plays a leading role in the clinical picture; As a rule, the dexamethasone test is negative. ACTH or corticotropin-releasing factor-secreting ectopic tumors are more common in men.

[11], [12], [13], [14]

Nelson's syndrome

On clinical manifestations is similar to the disease Itenko - Cushing. The appearance of ACTH-secreting tumors of the pituitary gland after bilateral adrenalectomy, performed for hyperplasia of the adrenal cortex, was detected. Appearing after adrenalectomy, increased corticotropin-releasing factor production and increased adrenocorticotropic activity in plasma may further promote ACTH-secreting pituitary tumors.

Madelung disease

The main manifestation of the disease is a pronounced local fat deposition in the neck region. Fatty pillow around the neck, gives the patients so specific a kind that a differential diagnosis is usually not required. Pathogenesis and etiology are not fully understood. They are referred to the cerebral form of obesity.

Treatment: sometimes excision of adipose tissue surgically is used.

The disease of Barraker-Simons (progressive lipodystrophy)

Only women are ill, usually after reaching puberty. The disease manifests itself in the uneven distribution of fat with the disappearance of the fat layer in the upper half of the body and the excessive development of fat deposits in the lower half of the body. Fat deposition is observed mainly on the hips in the form of a kind of "riding breeches." Weight loss of the upper half of the trunk and face are very pronounced. Symptoms of a disease of a symmetrical nature are more often observed, but asymmetric forms may occur, with the predominance of uneven fat distribution on one side of the body. Locally atrophic form of lipodystrophy is observed, as a rule, in diabetic patients at the place of insulin administration. There are reports of adherence to lipodystrophy of bilateral or unilateral facial atrophy with atrophy not only of the subcutaneous fat layer, but also of the muscles.

Of particular importance are hereditary factors and trauma.

Treatment of Isenko-Cushing syndrome

In the presence of an ACTH-secreting tumor of the pituitary gland, an appropriate surgical or radiation exposure is recommended. Pharmacotherapeutic agents use drugs that affect either the biosynthesis of cortisol in the adrenal glands (metapyrone) or the metabolism of neurotransmitters in the central nervous system (parlodel, cyproheptadine, reserpine). Parlodel is used in a dose of 4 to 7.5 mg / day. Within 6-8 months; cyproheptadine (peritol, deseril) - in a dose of 25 mg / day. Also a long time; reserpine - in a dose of 2 mg / day. Along with this therapy, traditional methods of reducing body weight are used.