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Persistent galactorrhea-amenorrhea syndrome: a review of information
Last reviewed: 23.04.2024
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The syndrome of persistent galactorrhea-amenorrhea is a characteristic clinical symptom complex that develops in women due to a prolonged increase in the secretion of prolactin. In rare cases, a similar symptom complex develops at a normal serum level of prolactin, which has an excessively high biological activity. In men, chronic hypersecretion of prolactin is much less common than in women, and is accompanied by the development of infertility, impotence, gynecomastia, sometimes with lactoremia.
The syndrome of persistent galactorrhea-amenorrhea has long been considered an extremely rare disease. Isolation of various forms of the syndrome, depending on the presence or absence of radiographically detectable adenoma or from previous pregnancy and childbirth (Forbes-Albright syndrome, Chiari-Frommel, Ayumada-Argonsadel Castillo) aggravated the erroneous assumption of its rarity.
In the early 1970s, thanks to the development of a radioimmune method for the detection of prolactin and the introduction of a polytomography of the Turkish saddle, it was found that the chronic hyperproduction of the pituitary prolactin accompanies every third case of female infertility and can be either the main pathogenetic link of an independent hypothalamic-pituitary disease, a number of endocrine and non-endocrine diseases with secondary involvement in the process of the hypothalamus and pituitary gland.
The term "persistent galactorrhea-amenorrhea syndrome" can not be considered as fully reflecting the essence of the disease. There is no statistical data on the actual frequency of hyperprolactinemic syndrome and its specific form-the syndrome of persistent galactorrhea-amenorrhoea. In the last twenty years, the number of diagnosed cases has increased significantly. Carried in 1986 by K. Miyai et al. Mass screening (surveyed 10550 residents of Japan, who did not make any complaints) revealed 5 patients with prolactinoma, 13 people with drug hyperprolactinemia and 1 patient with the syndrome of the "empty" Turkish saddle. It can be assumed that the frequency of prolactin, at least in the Japanese population, exceeds 1: 2800 in males and 1: 1050 in females. Data of autopsies reveal an even greater frequency of asymptomatic prolactin, but it is not clear whether these formations have any clinical significance.
The syndrome of persistent galactorrhea-amenorrhea is a disease of young women, extremely rare in children and the elderly. The average age of patients is 27-28 years. Significantly, the disease is diagnosed in men, usually at the age of 25-40 years, although cases of hyperprolactinemia in adolescents and in the elderly are described.
Causes and pathogenesis of the syndrome of persistent galactorrhea-amenorrhea
The genesis of pathological hyperprolactinemia is not uniform. It is assumed that the basis of the syndrome of persistent galactorrhea-amenorrhea due to the primary lesion of the hypothalamic-pituitary system is the violation of tonic dopaminergic inhibitory control of prolactin secretion.
The concept of primary-hypothalamic genesis suggests that a decrease or absence of the inhibitory effect of the hypothalamus on the secretion of prolactin leads first to hyperplasia of prolactotrophs, and then to the formation of prolactin in the pituitary gland. The possibility of persistence of hyperplasia or micropropactinoma, which does not transform into the subsequent stage of the disease (that is, into macroprolactinum - a tumor that extends beyond the Turkish saddle) is permissible. At present, the hypothesis of primarily pituitary organic lesions (adenomas), which is not detected in the early stages by conventional methods, dominates. This adenoma is monoclonal and is the result of a spontaneous or induced mutation, as the promoters of tumor growth may act as releasing hormones, multiple growth factors (transforming growth factor-alpha, growth factor of fibroblasts, etc.) imbalance between regulatory influences. Moreover, excess prolactin entails the production of surplus dopamine by the neurons of the tubero-infundibular system.
Causes and pathogenesis of the syndrome of persistent galactorrhea-amenorrhea
[Symptoms of persistent galactorrhea-amenorrhea syndrome
The most common reason for the treatment of women with persistent galactorrhea-amenorrhea syndrome with a doctor is a disorder of the menstrual cycle and / or infertility. The first varies from opsoligomenorei to amenorrhea, most often secondary. Polymenorrhea is not characteristic for hyperprolactinemic syndrome, except for its symptomatic forms associated with primary hypothyroidism. Approximately every fifth patient reports that menstruation has been irregular since menarche, the time of onset of which in many patients is somewhat delayed. In the future, particularly violent disorders of the menstrual cycle are revealed in the period of chronic stressful situations (examination session, long-term illnesses, conflict situations). The development of amenorrhea often coincides with the onset of sexual activity, the abolition of previously used oral contraceptives, the interruption of pregnancy, childbirth, the introduction of intrauterine contraceptives, or surgical intervention. As a rule, patients with the syndrome of persistent galactorrhea-amenorrhea are more concerned about violations of the menstrual cycle and / or infertility.
Galactorrhea is rarely the first symptom of the syndrome of persistent galactorrhea-amenorrhea (no more than 20% of patients) and even less often - the main complaint. Sometimes, even with a significantly increased level of prolactin, it is absent.
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Diagnosis and differential diagnosis of persistent galactorrhea-amenorrhea syndrome
If the diagnosis of the typical forms of the syndrome of persistent galactorrhea-amenorrhea today seems to be quite simple, the differential diagnosis of "erased", "incomplete" forms from the symptomatic forms of the syndrome of persistent galactorrhea-amenorrhea, as well as various vaguely outlined and poorly studied clinical syndromes in which galactorrhea develops against the background of normal serum prolactin level and its correction does not change the course of the underlying disease and does not alleviate the patient's condition, it is very complicated.
The laboratory and instrumental examination necessary to confirm the presence of the syndrome of persistent galactorrhea-amenorrhea consists of 4 stages:
- confirmation of the presence of hyperprolactinaemia by determining the serum level of prolactin;
- exclusion of symptomatic forms of the syndrome of persistent galactorrhea-amenorrhea (definition of the functional state of the thyroid gland, the exclusion of Stein-Levental syndrome, hepatic and renal insufficiency, neuro-reflex and drug effects, etc.);
- clarification of the condition of adenohypophysis and hypothalamus (radiography of the skull, computer or magnetic resonance imaging of the head, if necessary with additional contrast), carotid angiography;
- clarification of the condition of various organs and systems against the background of chronic hyperprolactinaemia (determination of the level of gonadotropins, estrogens, DEA-sulfate, study of the state of carbohydrate and fat metabolism, bone system, etc.).
Diagnosis of the syndrome of persistent galactorrhea-amenorrhea
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Treatment of the syndrome of persistent galactorrhea-amenorrhea
Drug therapy occupies the main place in the treatment of all forms of the syndrome of persistent galactorrhea-amenorrhea of the hypothalamic-pituitary genesis. With adenomas, it is supplemented or competing with neurosurgical intervention or with remote radiation therapy. Until the 1970s, SSTA was considered incurable. However, this idea changed after the introduction into the medical practice of the semisynthetic alkaloid ergot, parlodel (bromocriptine), possessing the properties of the hypothalamic and pituitary agonist dopamine (DA-mimetic), and also capable of inhibiting the growth of prolactin in some patients by influencing the genetic apparatus of prolactotrophs.
The sequence of application of various methods of treatment and their choice in each specific case are still debatable.
With the "idiopathic" form of the syndrome of persistent galactorrhea-amenorrhea, treatment with parlodel for the restoration of fertility, normalization of the menstrual cycle, elimination of sexual, endocrine-metabolic and emotional-personality disorders associated with hyperprolactinemia is indicated. If the concept of a single genesis of the disease is correct with the transition of "idiopathic" forms to a microadenoma, the application of the parlodel can have a prophylactic value.
Treatment of the syndrome of persistent galactorrhea-amenorrhea