What is Neuroendocrine Acromegaly?
Neuroendocrine acromegaly is a neuroendocrine disease caused by damage to the hypothalamus and (or) the pituitary gland and manifested by a pathological increase in the hands, feet, facial bones, enlargement of soft tissues, an increase in internal organs, thickening of the skin, and metabolic disorders.
The emergence of acromegaly is caused by excessive release of somatotropic hormone (growth hormone) into the blood during a hormonally active tumor (pituitary adenoma) or increased secretion of the releasing factor of somatoliberin growth hormone by the hypothalamus after craniocerebral trauma, acute or chronic neuroinfection, or trauma, and neuroinfluency. patients with acromegaly also note elevated blood levels of prolactin. The disease develops equally often in women and men aged 20 to 50 years.
Symptoms of Neuroendocrine Acromegaly
Neuroendocrine acromegaly is characterized by a combination of symptoms caused by an excess of growth hormone (sometimes prolactin) and a lack of other pituitary hormones, as well as symptoms of increased intracranial pressure and compression of the optic nerves by a growing tumor. The disease develops gradually. There is a change in the appearance of patients. Their facial features become rough, the superciliary arches, nose, lower jaw increase, the interdental spaces widen, the bite is broken. A progressive increase in feet and hands, especially in width, forces patients to use shoes and gloves of large sizes. In patients with acromegaly, spinal curvature, an increase in the size of the chest in the anteroposterior direction (barrel-shaped chest) are noted. Arthrosis often develops, gross hairiness appears, the skin thickens and forms folds, and excessive sweating is characteristic. An increase in the larynx, tongue (macroglossia) and vocal folds leads to a change in voice: it becomes hoarse and low. An increase in the size and mass of internal organs (splanchnomegaly) is accompanied by a violation of their function – heart failure develops, blood pressure rises, clinical signs of liver dystrophy and pulmonary emphysema appear. Thyrotoxicosis and the appearance of nodes in the thyroid gland are characteristic of the initial period of the disease. Later, with a large pituitary tumor, hypothyroidism and adrenal insufficiency are noted, which is manifested by increasing muscle weakness and edema. In half of the patients, glucose tolerance is impaired, signs of diabetes are found. Almost all women under the influence of an excess of prolactin and a lack of gonadotropic hormones disrupt the menstrual cycle, often there is a release of milk from the mammary glands, not associated with pregnancy and childbirth – galactorrhea. One-third of men have decreased sexual potency. Sometimes the secretion of antidiuretic hormone decreases, which causes the appearance of signs of diabetes insipidus. As the pituitary tumor grows and intracranial pressure rises, constant headache, vomiting, dizziness, and visual disturbances occur.
When the appearance of characteristic changes in appearance, which gives reason to suspect acromegaly in a patient, he should be immediately sent for a consultation to the doctor. Further examination is carried out in a specialized hospital.
The prognosis for life with a timely diagnosis and proper treatment is favorable, there may be some improvement in appearance by reducing the volume of soft tissues, although cosmetic defects are not completely corrected. With large, rapidly growing inoperable tumors and severe disorders of the function of the cardiovascular system, the prognosis is poor. Patients with acromegaly should always be under the supervision of an endocrinologist, an ophthalmologist and a neurosurgeon.
Diagnosis of Neuroendocrine Acromegaly
The diagnosis is established on the basis of the characteristic appearance of the patients. It is confirmed by a high concentration of growth hormone in the blood and the results of an x-ray examination of the skull and skeleton. The size of pituitary adenoma (if it is the cause of increased growth hormone formation) and the nature of its increase is judged by radiography of the skull in the dynamics, computed tomography of the head, as well as on the basis of the fundus picture and determining the state of the visual fields.
The differential diagnosis is carried out with Paget’s disease, accompanied by specific deformity of the long bones and the cranial vault.
Treatment of Neuroendocrine Acromegaly
Treatment for a pituitary adenoma that has been identified consists of surgical removal of the tumor or its radiosurgical destruction. If it is impossible to have surgery or the ineffectiveness of radiation therapy, the long-term use of parlodel is a drug capable of suppressing the secretion of growth hormone. Symptomatic treatment is aimed at reducing intracranial pressure, etc.