EUNUCHOIDISM

From Big Medical Encyclopedia

EUNUCHOIDISM (Greek eunuchos the eunuch, skopets + eidos a look) — the clinical syndrome caused by hypofunction of gonads and which is characterized by an underdevelopment of sexual characters, a disproportional constitution (rather shortened trunk, long extremities with a high growth), it is frequent obesity.

The term «eunuchoidism» is offered by Griffith and Dakuort (Griffith, Duckworth), in a wedge, terminology is entered in 1913 by Yu. Tandler and S. Grosz.

E. meets seldom; at women there is a lot of less than at men.

Etiology

E. as the syndrome is manifestation of a number of the states connected with insufficiency of gonads: inborn (see. Anorkhiya , Hypogonadism , Dysgenesis of gonads etc.) or acquired during the dopubertatny period (an injury, surgical castration, inflammatory process, a new growth, etc.).

Pathogeny

E. is defined by deficit in an organism of the sex hormones regulating development of generative organs and providing formation of secondary sexual characteristics and normal functioning of many bodies and systems. Weakening of synthesis and secretion of hormones can be caused as primary pathology of hormone-producing elements of a small egg or an ovary, and disturbance of gonadotropic function of gipotalamo-pituitary system. Insufficiency of gonads in the dopubertatny period leads to disturbance of their germinative function, an underdevelopment of generative organs and secondary sexual characteristics, disbolism and proportions of a skeleton (due to delay of closing of epiphyseal zones and, as a result, increase in duration of growth of bones).

Pathological anatomy

Pathoanatomical changes at E. touch first of all gonads. Testicles are often localized in an abdominal cavity or the inguinal channel (Cryptorchism).

Find vitreous degeneration of a basal membrane in seed tubules; cells of seed tubules are small, the spermatogenesis is absent, glandulotsita (a cell of Leydiga) are poorly developed. Appendages of testicles are usually not changed. The ovary is small, dense, sclerosed, the white is thickened. Process of maturing of follicles is broken. The uterus and a vagina are underdeveloped. The hyperplasia of goitrous gland, adrenal glands, a thyroid gland, sometimes small expansion of the Turkish saddle is found. Limf, nodes also giperplazirovana. Epiphyseal zones of ossification are not closed. At men the basin has the form of women's.

Clinical picture

Fig. 1. Youth eunuchoid of 19 years with a characteristic disproportion of growth: long extremities at rather short trunk. Fig. 2. Male eunuchoid with a characteristic type of obesity of a breast in mammary glands and the bottom of a stomach. Fig. 3. The girl eunuchoid with a characteristic disproportion of growth and obesity of the bottom of a stomach.
Fig. 4. The person of eunuchoid — men. lack of vegetation on a face.

Expressiveness E. considerably depends on at what age the disease began. Picture typical E. gives only dopubertatny development of insufficiency of gonads. The child becomes more senior, the signs E are shown more clearly. Along with sharp disturbance of sexual development, extremely poorly expressed secondary sexual characteristics for E. the disproportion of growth and obesity is characteristic (fig. 1 — 3). In some cases dominance of a disproportion of a skeleton — long extremities can take place at rather short trunk, in others — obesity, in the third — a combination of that and another. The adiposity is quite typical: on a breast in mammary glands, in the bottom of a stomach, in the field of a basin, on a pubis. The expressed eunuchoid obesity occurs at children and young men less than at adults. Skin is wrinkled, yellowish (geroderma), skeletal muscles are developed poorly. External genitals are underdeveloped, secondary sexual characteristics, in particular pilosis, or are expressed very poorly, or completely are absent (fig. 4). If male eunuchoids have a pilosis, then it gets female type. The voice usually remains women's, the throat is underdeveloped and the thyroid cartilage does not act. Patients usually have no sexual feeling. The condition of generative function is defined by a form of a hypogonadism, typical eunuchoids are fruitless. Changes of cardiovascular system (small heart, a narrow aorta), decrease in the ABP are noted.

Mental disorders at E. are usually expressed unsharply and practically do not influence working ability of patients and their social adaptation.

Specific psychoses at E. do not meet. At careful and long observation at patients some features which are keeping within asthenic option of an endocrine psychosyndrome can be noted: increased fatigue and sensitivity (sensitivity), shyness, shyness, quite often instability of mood (see. Endocrine mental syndromes ). Suggestibility, tendency to imagination and some other lines of infantility can be observed. These features are expressed in different degree and meet not in each case. The depressions developing at some patients sometimes proceed with the ideas of the relation and morbid depression. In most cases depressive reactions are psychogenic. Do not make an exception in this respect and the reactions developing in certain cases in the postkastratsionny period. Noted features essentially differ from described in old references (decrease in memory and intelligence up to a deficiency of intellect, epilepsy and hysteria at eunuchoids, the lowered sincere vivacity, emotional coldness up to cruelty, insidiousness, cowardice, laziness, etc.). Contradiction in the description of mentality of the patients suffering E., it can be explained not only with an inaccuracy of judgments of some authors and casuisticity of separate observations, but also the pathomorphism of the disease caused by progress of hormonal replacement therapy, and also changes of social conditions.

The diagnosis

the Diagnosis usually does not represent difficulties. At differential diagnosis it must be kept in mind all those a wedge, forms which are characterized by a sharp delay of sexual development, and in particular adiposogenital dystrophy (see), at a cut quite often find an intrasellyarny tumor of a brain appendage.

The disproportions of a skeleton characteristic for E., at an adiposagenital syndrome does not happen. At an underdevelopment of generative organs pituitary dwarfs have a sharp growth inhibition, at E. — usually its acceleration.

The hypercholesterolemia and p-lipoproteinemiya is sometimes noted. Tolerance to carbohydrates is a little increased. The high catabolism of proteins is reflected in the raised excretion of nitrogenous compounds. The maintenance of androgens and estrogen in blood, excretion of 17 ketosteroids with urine are lowered.

Treatment

Treatment comes down to use of gonadotropic drugs and especially sex hormones. Girls and women have a use of drugs of sex hormones (estrone) or their synthetic substitutes (hexestrol, diethylstulbestrole, etc.) shall be carried out always under observation of the gynecologist. At men use of drugs of male sex hormones is effective (Testosteroni propionas).

In cases E. at boys and young men it is possible to be limited to use of methyltestosterone (1 — 2 tablet on 0,005 g, under language in the morning and before going to bed); at hypogonadotropic E. the good effect gives horiogonin. Adults need to appoint Testosteroni propionas intramusculary daily on 25 mg in 1 ml of oil solution or on 50 mg every other day. The monthly courses of injections conducted quarterly give therapeutic effect: the generative organ increases in sizes, secondary sexual characteristics clearly come to light. Weight at eunuchoids with big adiposities under the influence of sex hormones begins to fall slowly.

Treatment of mental deviations at patients E. in most cases symptomatic (antidepressants, etc.), the rational psychotherapy shall figure prominently.

The forecast

the Forecast for life favorable. Treatment usually does not come, but under the influence of prolonged use of hormonal drugs patients reach manhood and make an impression of healthy men, in many cases — become capable to sex life. Infertility at E. practically it is not curable. The forecast is more favorable at a secondary (hypogonadotropic) hypogonadism.


Bibliography: Liberman L. L. Inborn disturbances of sexual development, L., 1966, bibliogr.; To Milk Sh t. - M and Denile-Muster A. Gynecologic endocrinology, the lane from Romanians., Bucharest, 1973; The Multivolume guide to internal diseases, under the editorship of E. M. Tareeva, t. 7, L., 1966; It is old kovan.t. Fundamentals of clinical andrology, M., 1973; Teter E. Hormonal disturbances at men and women, the lane with polsk., Warsaw, 1968; L a b h and of t A. Klinik der inneren Sekretion, B. u. a., 1971; Textbook of endocrinology, ed. by R. H. Williams, Philadelphia, 1974.

H. A. Shereshevsky, D. D. Orlovskaya (psikhiat.).

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